narayanapediatriccardiology

2011-07-15T16:34:00.001+05:30

This is Dr Kiran welcoming all to the new post of the blog.

This time, the delay in the posting was not my usual laziness, but it was because of some tight rope walking! It was arrival of my second baby. He was in a hurry and the delivery happened 18 days prior to the due date. We were obviously not well prepared and had to do some circus to get the things settled. Now that all iz well, I am posting this update on the blog. Hope I will get some comments this time, for right or wrong reasons!!

VOLUME Vs PRESSURE

I think we have discussed this issue earlier, but without much clarity. I wish someone replies this time. In cases of single ventricles with borderline high PA pressures, is the recommendation of PA tightening valid? One side of the logic is, by tightening the PA, flow into PAs would be lesser and thereby the pressure. Also, adding BD Glenn shunt in this situation would improve the saturation and off-load the ventricle.
On the other hand, tightening the PA would increase the afterload on the single ventricle and would nullify the effect of offloading the ventricle. Also, when the baseline PVR itself is less (baseline PA pressures in range of 18-20mmHg), what more improvement would PA tightening achieve in PVR/SVR ratio? When the long term threat is failure of single pump, is volume load better or pressure load?
Please let me know your takes on this issue.

THE NUMBER GAME

Sometimes, we decide to cath some borderline cases for objectiveness and end up cursing our decision to cath!! We had one-and-a-half year old with ASD and VSD with severe PAH, with saturation of 93%. His LV looked non-compacted and the extent of pulmonary hypertension was higher than expected for the extent of lesions. One of the consultants thought of acquiring more data on the issue and decided to cath. Data on cath showed a PVRI of 11wood units! Since the child had all the features of operability on clinical evaluation and baseline investigations, these numbers looked shocking. It is not easy to convince the surgical team to operate when the cath produces such results! However, one of the senior surgeons was kind enough to ignore the cath data and operated the baby! The learning was: when in doubt, don’t just cath. You may get unexpected turn in the way!! Please share your experiences in this issue.

END DIASTOLIC PRESSURES AND OPERABILITY

Many places follow the rule of diagnostic cath in Down syndrome babies with congenital heart defects older than 1 year. We had a 2-year-old baby with Down syndrome, who had a large inlet VSD. Although operability on clinical grounds was never an issue, it was sought by someone as ‘knee-jerk’ response. The LV EDP was documented to be 25mmHg. The tracings were verified and found correct. Barring that, the data on operability showed operability. One of the senior surgeons showed his reluctance in operating babies with high EDP. Our recommendation of leaving an ASD open also did not go well. What is the take of readership? Is high EDP a detriment in surgery? Can you recall having seen a similar situation and outcome of that baby? Please share your experiences and expertise in this.

DEVIL Vs DEEP BLUE SEA

As a matter of convention, I have heard many centers have different PVRI cutoffs for ASD and VSD operability. One convention was: less than or equal to 7 wood units for VSD/PDA and less than or equal to 10 wood units for ASD. The question was- when the PVRI is between 7 and 10 wood units in cases of VSD, can we convert the physiology into an ASD? In other words, can we close the VSD and create a sizable ASD in that patient? On long run, the progress of disease might be better with a pre-tricuspid shunt than a post-tricuspid one. However, our surgical team opined that giving another disease for one even after a major heart surgery is useless and worthless. Are there any studies on these lines? What would your personal opinion on this be? Please let me know. I would be interested if this issue can raise a debate.

RIMMING THE FLOW

In cases of toddlers with moderate to large ASD and near normal PA pressures, how frequently would one witness cyanosis? Even the sinus venosus defects of SVC type where the SVC straddles over both atriae, we rarely see cyanosis. I have seen 3 cases of sinus venosus defect of IVC type and not even one of those three had cyanosis. However, my experience in the latter is obviously less. It is said that a long, redundant Eusthecian valve can some times direct the IVC blood towards LA in low secundum ASDs with deficient IVC rim. In the absence of systemic venous anomalies and unroofed CS, is it possible to have cyanosis in ASD with normal PA pressures? We had one of such kids in our hospital. Conventional and saline contrast echo was not useful in deducing the picture. Cath study is unlikely to yield much. How would you go about in such cases? Please share your experiences on this.

That brings us to the end of the present post. Please send your criticisms and suggestions typed in the comments box or directly to my email: drkiranvs@gmail.com If you wish to contribute your experiences, please type them in a word document and send the attachment to my mail. I shall publish them on your behalf with full credit to the writer! See you shortly with few more learning scenarios.

Regards

Kiran

2011-06-16T08:33:00.000+05:30

Dr Kiran welcomes you to the new post of blog.

It is often told that thinking is easier than writing! However, I have found the contrary to be true. In the past 30 odd months of single focus blogging, I have found better expression with writing than verbalizing the same issue. All this is to tell the readership that writing comments is good – for yourself and for the blogger!!

Let us dwell into the interesting learning scenarios right away:

WHY COLLATERALS?

In post-Glenn status, it is not uncommon to find venovenous collaterals. How common is the development of new aorto-pulmonary collaterals during post-Glenn? We had an 11-year-old girl with such a picture. Her pressure data was normal and she also had good-sized branch Pas. However, she had leash of new aorto-pulmonary collaterals which was not found during the previous cath study. We know the impetus for venovenous collaterals – usually the dissipation of pressure. What is the impetus for new AP collaterals when the pulmonary arteries and saturations are OK? Is there any other impetus for development of new AP collaterals other than cyanosis? To best of what we have seen, this finding is infrequent. I would be interested in finding experiences and inputs from readers.

NEED OF HOMOGRAFT

This was a good learning. We had a 6-year-old with Tetralogy of Fallot with absent Right Pulmonary artery. On cath, we found the right lung supplied by three high pressure collaterals from thoracic aorta at T5-6 level. An innovative looking surgery was suggested by our senior consultant – resecting the collateral portion of aorta en masse, unifocalizing that segment with existing pulmonary artery and interposing a graft for the resected aortic segment. However, the surgical team was not keen on this, as the complexity of surgery would be higher for no better yield. It was decided that the lesion is overall repairable with unifocalization and intracardiac repair. The question was the need for homograft. It was successfully argued that the unifocalized high pressure collaterals would require competent valve. A transannular patch in such cases would result in torrential regurgitation due to high afterload. It was a good “retrograde” learning!

STABLE BLUE BOY

When we discuss the differentials of hemodynamically stable, asymptomatic blue toddlers, we often describe the utility of murmur. The general rule is, better the murmur – stabler the patient, as the bottomline in these cases are extent of pulmonic stenosis. For example, a toddler with tetralogy of Fallot with good murmur over left upper sternal border has better hemodynamic stability than the one without murmur. For, murmur indicates blood flow across the narrow RVOT. The exceptions to this rule are few. We had a toddler with uniform saturation of 76% in all limbs. He was totally asymptomatic- barring the only complaint: cyanosis. The arterial blood gases showed low pO2 and marginally high pCO2. His physical examination did not reveal any significant finding. Cyanosis and clubbing were confirmed. He had normal heart sounds and no murmur. Chest radiograph was normal. There was no reason to suspect cardiac cause of cyanosis. On echo, we found the issue. The SVC was opening totally into LA, without any ASD! There was no LSVC. IVC was normally into RA. All the pulmonary veins were opening into LA. Surprisingly, the LA and LV dimensions were normal. How these tougher chambers accommodated the extra blood without getting dilated was enigmatic! This was the first time I saw a partial anomalous systemic venous connection. I had earlier seen Raghib complex, but this one was new. The management is simple, but the presentation was puzzling and findings were not in mould! After a painful delineation of diagnosis, we found an outside report wherein they had not only diagnosed the problem, but had also done a saline contrast injection in the upper limb, documenting the entry of SVC flow into LA. Good show indeed! We searched the literature and found only 19 reported cases so far. Most of them had associated anomaly. This is probably one among very few cases of isolated RSVC to RA without any other cardiac anomaly.

BREEDING THE NEED

This is not the problem of one centre; virtually every centre I have come across have this problem – some more some less, but inevitably, yes. I am talking about the unnecessary diagnostic cath studies performed in our country. It is accepted that Pediatric cardiology is still young field and rigid consensus are not possible yet. But, many times, those children who have their decision written very well on clinical grounds end up getting diagnostic cath studies. Whether it is ignorance or felt-need or greed or breeding the culture of teaching the evidence to trainees is questionable. We do come across patients carrying reports and CDs wherein the cath study was done for absolutely useless indications. Consider these examples: 7-month-old child with failute to thrive, saturating 98% in room with large VSD. Another child with truncus arteriosus where one cannot get either the numerator or denominators of the equations to calculate cath data. Worse of the lot is when the consensus is made based on chest radiograph and ECG after a thorough cath study! Isn’t it possible to create an institutional consensus on the need for cath? Many centres do a combined cardiologist-surgeon cath meetings to discuss the decisions. Isn’t it time to do a pre-cath meet to decide the utility of cath study in individual cases? I strongly feel that everyone owes the responsibility of passing on scientific knowledge to those who look upon us. I have tried to bring up this issue many times, but the futility is obvious every time. I would like to hear experiences and thoughts of readers on this issue.

FATE OF THE UNPAIRED

I have brought up this issue once in the past, but this time it was bigger. We evaluated a 22-year-old DORV, VSD, PS who had undergone a BD Glenn shunt at an outside hospital in 2009 (at age of 20 years)! On enquiry, we understood that the earlier centre had planned a two-pump repair on her, but found the VSD to be non-routable. Hence, they took on table PA pressures and performed a Glenn shunt instead. However, she came back with no improvement in saturations or effort tolerance. Her baseline SO2 was 70%. We did the cath study in our centre. Her PA mean pressure was 12mmHg. However, we found a hugely dilated azygous vein entering the SVC. We balloon occluded the azygous to notice the increase in PA mean pressure to 14mmHg. Her IVC was not interrupted. Her EDP was 10mmHg. The question was: why did the azygous vein dilated so much? The lower body is well drained by the IVC. The PA pressures are acceptable. How did the azygous dilate at all? Normally, the azygous vein gets ligated during BD Glenn shunt. But here,http://www.blogger.com/img/blank.gif the previous centre had not done that. It is true that the azygous would behave like a pop-off, but can it dilate so much in the absence of any impetus? Is it only the pro-gravity phenomenon wherein the SVC blood finds it easier to drain into azygous than to PA? Are there venous valves in azygous? Please let me know your experiences on this.

That brings us to the end of present post. Please pen your criticisms and suggestions in the comments box or direct them to my email: drkiranvs@gmail.com See you shortly with few more learning scenarios.

Regards

Kiran

2011-05-31T17:19:00.000+05:30

Dr Kiran welcomes you to the present post of blog.

As usual, I did not receive any comments for the last post. But I have heard that many people do read and follow the blog. Just that they are as lazy as I am when it comes to writing!

Without much ado, let us carry on with the interesting learning scenarios:

SMOOTH FLOW PALLIATION?

We had a 6-year-old with congenitally corrected transposition of great arteries, with small VSD (non-routable) and severe subvalvar and valvar PS. The LV was suprasystemic and TR was moderate. Neither double switch nor Senning-Rastelli were possible. The surgeons were not happy with the tricuspid valve and ruled that the conventional repair (RA to LV to PA and LA to RV to Ao) by pulmonary valve repair was inadequate unless the TV was also intervened. However, their scepticism was, “what if the TV is not repairable?” The child was too young for TV replacement. We argued that the pulmonic valve repair alone should bring down the LV pressures and improve the LV geometry. This would take away the Bernheim-reverse Bernheim effect and would improve the RV geometry in return, thereby reducing the TR. I felt this intervention would be better than leaving a suprasystemic chamber for follow up. But, this logic did not please the surgical team and they ruled out any such improvement. Although it sounds theoretical, I could see practical point in it. How would the readers like to decide? Please let me know the folly in this logic!

INSIDE THE HEART OF FONTAN

Once a single ventricle goes into the Fontan correction, we usually feel that the woes for patients lie outside the heart. We had a 12-year-old with Fontan completion done 5 years back. This child came with a new cardiac murmur, not documented in the previous records. On echo, we found a significant subaortic obstruction! I had not come across this earlier. This actually poses a new issue for the patient. Since the obstruction was moderate, the surgical team suggested close follow up. If this becomes severe, what would be the plan? Is going inside the Fontanised heart simple? How frequent are such complaints? Our experience with Fontan is not huge. Can anyone with more experience help us in this?

MONOCUSP

We had some heated debates and discussions on the utility of monocusp valve in intracardiac repairs of tetralogy? Few argued for long-term benefits and few for short-term. Surgeons discussed the risk-benefit ratio of monocusp valve. However, at the end of all these, no one was ready to change their views and opinions. That left most of the simple audience with more confusion and no conclusion. Can the readers put up their opinions in this issue and get us better understanding?

FONTAN WITH HEART BLOCK

Sinus rhythm is one of the Choussat’s criteria for Fontan completion. We had a 9-year-old boy with BD Glenn shunt done outside 5 years back. He had NYHA class III symptoms. During OPD evaluation, he had an ECG which documented sinus rhythm. He was catheterised and the pressures were OK for Fontan completion. However, in the ward, we documented slow heart rate and Holter monitoring showed intermittent A-V dissociation. Surgeons opined that the Fontan completion is still possible with DDD pacemaker insertion simultaneously. This was news to us. Is it done? Is it acceptable? Please let us know about your opinion and experience.

STUBBORN PA

We had a 6-year-old who had undergone a PA banding for multiple VSDs at an outside hospital few years back. The same hospital did a PA de-banding and closed a large upper muscular VSD at the same setting about one year back. When this child came to us, we were surprised to find few sizeable VSDs and a significant gradient across the MPA segment. The operative notes at outside hospital had clearly stated PA de-banding. But, our echo could still pick up a constricted segment in the MPA prior to bifurcation. On cath, we found this narrow segment, which had cicatrised and did not re-open following de-banding. As for the residual VSDs, our expert surgical team was confident that all of them can be dealt with. Hence, we decided for a resection of cicatrised MPA segment and closure of VSDs. How common is this cicatrisation at PA band level? Although we perform good number of PA bands, we at NH had never witnessed this complication. Are such lesions innate to few patients or is it widely seen? Please let us know your experiences on this.

It is time to congratulate Dr Shwetha Nathani, our fellow for the last two years for having successfully completing the Fellowship FNB exam in Pediatric Cardiology. All the good luck for her bright future.
It is time to mourn another departure. Our beloved echo technician, Mrs. Harini is moving to Chennai city for good. We all miss her expertise and keen acumen. Good luck wherever you go.

Pen in your criticisms at comments box or to drkiranvs@gmail.com Shall get back shortly.

Regards

Kiran

2011-05-15T21:37:00.001+05:30

Oops!
Five months that I have updated anything in the blog!!
Sincere apologies.
I know lot of readers have stopped checking the blog. I know I have been unfaithful!!
There were times of busy deadlines, extracurricular works, depressions, blocks, fights, heart-breaks and so on. But none of them can explain the actual reason for the absence of updates: It is pure laziness on my part.
Also, lack of responses from the readers was negative impetus.
One of my teachers suggested me: do it for yourself and be happy if few more get benefitted.
But, showtime spirit is not in favour of it!!
I figured out few ways I can be more consistent. First was restructuring the blog.
I shall continue this blog with pure Pediatric Cardiology material. Shall try to make it more dense and focussed. It should serve for those into this subject or have serious interest in the field.
That actually takes away a substantial amount of readership, mainly – paediatricians. To compensate, I had to think of few ways out:

1. Proposal to start an exclusive interactive blog on Pediatric cardiology for Pediatricians. In this, we would touch common cardiac problems of children which are of much interest and frequency to paediatricians. This blog which is shaping up at present, tries to make an interactive forum with readers, inviting questions from them and giving answers, putting up practical tips on diagnosis and management. I shall give the info shortly.
2. Got a proposal to start one book-club blog! This would be pure non-academics. It aims at getting the readers of blog to write on what interesting non-academic work they have read. It can be a present reading or even a book that had a large impact on them in the past. Purely literature and anything other than politics and religion!!

Working on these ideas have got me an excuse not to write for the present blog. But, I could not avoid the temptation of keeping this blog alive. Hence, I aim to continue this with as much precise info as possible.

I have cut away the segments on articles, reviews and pearls. So, what remains in the blog is purely interesting learning discussions. I shall try to update them as and when possible.

With this prelude, let me continue to the main segment: Interesting learning scenarios:

PRESSURE AND POWER
It is often said that the left ventricle in Ebsteins anomaly gets jeopardised due to innate structural reasons and lateral pressure from atrialised RV. Can the low pressure atrialised RV exert sufficient power to deform the mightier LV? Is the phase of cardiac cycle in which the events occur responsible for such behaviour? If there is space compromise, wouldn’t the low pressure chamber suffer more than the high pressure one? What is the take of readership on this? Please let me know.

DEVICE REGURGITATION
What is the incidence of aortic valve regurgitation following VSD device closure? I would presume it is rare. The scepticism on perimembranous VSD devices are always to do with conduction blocks than aortic valve. Also, it is largely in the case selection. We are always concerned about the distance between VSD and the valves. If a perimembranous VSD device has resulted in mild+ AR, how should it be tackled? Should we just follow it as any other AR, watching for progress and intervening at the right time? Or, should we be aggressive because we have a prosthetic material impinging on the valve and deforming it? If the readers have come across such instances, please enumerate your experiences for others. Otherwise, what is the logical conclusion? We wanted to wait, but surgeons advised us to get the device out. What would your say be?

WHATS IN A NAME?
We had an interesting observation. It was a 5-year-old asymptomatic child with bilateral SVC. However, the LSVC showed flow away from the heart and RSVC was into the heart, with a proper H-communication. The pulmonary veins were normal and all of them were draining effortlessly into LA. We ruled out TAPVC, PAPVC and levo-atrio-cardianal vein. So, what explains the reversal of flow in LSVC. On careful review echo, we could trace the reversal till the H-communication (innominate vein), but the flow from the other end of LSVC was normal till the innominate vein! So, it is as if the innominate vein receives something from heart, apart from its own flow from head and neck. We traced the LSVC into the heart. It used to get blurred by the time we reached the RA. Finally we could figure out that the opening of coronary sinus into the RA was atretic! The cardiac venous flow came into coronary sinus and had a parallel vent in the form of LSVC. Thus, coronary venous flow ascended via LSVC, reached the incidental H-communication, went into the RSVC to eventually enter the RA, unobstructed! Our surgeons claimed that such lesions do exist and they have seen them earlier also. They also claimed that such a lesion has a name. We could not get this eponym. If anyone knows what this combo is called, can you please enlighten us? We did nothing for this patient, as the kid was totally symptom-free. Should we do something? Please let us know your opinion.

EDP IN DISTAL BLOCKS
How pertinent is the EDP in the presence of distal obstruction? We had a 4-year-old with moderate ASD and pulmonic stenosis. One of our consultants felt that the RV not dilated upto the expectation and advised cath study. In the cath, mean gradient across the pulmonic valve was 50mmHg, but the RV EDP was 18mmHg. I was not sure if this EDP reading should be given importance when there was a distal obstruction. Finally, the advice was to get a BPV done and re-assess the ASD. What is the yield of cath pressure data in the presence of such combination lesions? Please let us know your ideas on this.

STEALING BY FISTULA
We do come across few cases of coronary-cameral fistula in young children. We generally go by the volumes and make a decision whether it should be intervened or not based on the chamber dilatation. Do these fistulae cause coronary steal? How do we know it in younger children? Is thallium study useful when there is no wall motion abnormality on echo? I have rarely seen anyone deciding these lesions giving weight to potential steal. Please let us know your take on this.

Let us meet again with another five learning scenarios in short time. Before parting: welcome to our new NH Pediatric Cardiology fellows: Dr Arun Bableshwar, Dr Rahul Saraf, Dr Devprasath and Dr Kshitij Sheth. Hope you will have exciting learning and formative phase as pediatric cardiologists at NH. Also, good luck to Dr Shwetha Nathani – for her fellowship exit exam this month end at Escorts, New Delhi. We wish her the same success as she had for gold-medal winning case-presentation performances at various centres across India.

As usual, please put down any suggestions to drkiranvs@gmail.com or in the comments box

Regards

Kiran

2010-12-21T22:52:00.001+05:30

This is Dr Kiran welcoming the readers to the present post of NH pediatric cardiology blog. As discussed in the previous post, a new addition was to be made to the blog. After thinking for quite some time, I came up with this idea. A pediatric cardiologist is always in need for the current updates on the management of medical problems faced by the patient community. Seldom have we seen the busy practitioner sparing time to update himself on these issues. I have seen some of the very big names in the field erring badly in scientific sessions, not for the lack of knowledge but for the lack of updating it. I would be dealing with the current update in the management of some of the critical issues in our field. Let me get some feedback on this attempt, so that I can decide whether to continue this segment or not.

To begin with, let me touch upon Pulmonarty atresia with VSD with MAPCA dependent pulmonary circulation: The current concepts and dilemmas.

Some of the special characteristics of MAPCAs for revision:
• Derived from Splanchnic vascular plexus (DeRuiter et al in journal Circulation in 1993, titled ‘Development of the pharyngeal arch system related to the pulmonary and bronchial vessels in the avian embryo: with a concept on systemic-pulmonary collateral artery formation’)

• Intracardiac defects are fairly simple in MAPCA dependence rather than vertical PDA.

• MAPCAs are highly variable in their size, number, course, origin, arborization and histopathologic makeup. (Liao P et al in 1985 in JACC as ‘Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect’)

• The natural history of major aorta pulmonary collaterals includes progressive stenoses and occlusion, sometimes making any segment of lung supplied by these collaterals inaccessible or unusable if incorporated into definitive correction.

• Manipulation of these aorta pulmonary collaterals through attempts at unifocalization can produce iatrogenic occlusion in the form of scarring or anastomotic stenoses.

• Major aorta pulmonary collaterals without obstruction can lead to pulmonary vascular obstructive disease in the segments supplied by these collaterals.

• No intervention is needed for cyanosis in the neonatal period and in the overwhelming majority of patients, any attempt to manipulate the collaterals early in life is usually disastrous.

Some of the strategies for the management:

Unifocalization:

• Haworth and McCartney in 1980 published ‘Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries’ in the British Heart Journal. They conceptualised the unifocalization or bringing together of the abnormal collateral segments to ultimately form a pulmonary arterial confluence through a staged approach. However, the results with the staged approach of unifocalization have been extremely variable, ranging from 12 to 60% in various studies.

• Reddy et al in 1995 published ‘Midline one-stage unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals’ in JTCS. They opined that only 20–30% of a cohort of newborn infants with this combination of anomalies will have complete repair with acceptable right ventricular hemodynamics if a delayed staged approach is taken. These authors reasoned that a single stage correction in early infancy before stenoses in collaterals could occur, and before irreversible pulmonary vascular destructive disease could occur, would logically be an ideal strategy for managing this otherwise frustrating condition. It was reasoned that the earlier in life that the greatest number of healthy lung segments could be incorporated into the unifocalized pulmonary circulation, the more likely one is to capitalize upon the tremendous potential for additional growth and development of the pulmonary circulation and parenchyma that exists in early infancy.

• The single stage unifocalization as proposed by Reddy et al was achieved in much younger age group by Lofland. In a study titled ‘The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy’ published in European Journal of Cardiothoracic Surgery in year 2000, eleven consecutive infants over an 18-month period ranging in age from newborn to five months went through the protocol with great success.

Growth of Native PAs

• In 1991, Iyer and Mee published their study titled ‘Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals’ in ATS. The study strongly proposed promotion of growth of central pulmonary arteries with a shunt, either a central shunt or a direct aorta to native pulmonary arterial anastomosis.

• Duncan et al continued the hypothesis in 2003 JTCS article titled ‘Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries’. They proposed intervention beginning at approximately six months of age with the creation of a central shunt or a direct aorta to pulmonary arterial anastomosis.

• Ishibashi et al in 2007 published ‘Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries’ in European Journal of Cardiothoracic Surgery.

• Mumtaz et al in 2008 brought out their study in ATS titled ‘Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries’. Both the articles showed good results even in staged procedures.

• Griselli et al in 2004 had observed the relationship between the size of native PAs and the surgical outcome. In their article titled ‘The influence of pulmonary artery morphology on the results of operations for major aortopulmonary collateral arteries and complex congenital heart defects’ published in JTCS, they evaluated 164 patients over an 11-year period. A 69% complete repair was achieved. Most importantly, the best prognosis was with intrapericardial pulmonary arteries that were at least 75% of predicted size. The worst prognosis was in patients with no native pulmonary arteries, and the entire pulmonary blood supply supplied by collaterals only. There were no survivors at 10 years in this patient subgroup. Patients with diminutive but present native pulmonary arteries had an intermediate survival.

How can one go about:

Pulmonary atresia with VSD is a relatively less common entity. Hence, unless the centre is doing a high volume work, it may be difficult to get substantial numbers to bring up a management algorithm. The approach is more likely to be case based, as the possible variants are multiple. Yet, it may be possible to bring about the general guidelines in this issue. Let us see few tips on how to go about. This segment is open to additions from the readership. Please feel free to add on your views via comments box or by emailing me at drkiranvs@gmail.com. I shall moderate the additions as per need.

• The chief determinant of outcome is the provision of proper pulmonary circulation. Hence, good sized confluent pulmonary arteries carry the best prognosis. This is followed by potentially unifocalizable subset. To determine the pulmonary anatomy at any given age, Cardiac CT with 3D reconstruction can be very helpful. This modality shows how the collaterals course relative to other important mediastinal structures such as the trachea, esophagus and main stem bronchi.

• The next step would be a complete unifocalization as early in life as possible. The rationale for an early complete unifocalization is that there should be early normalization of cardiovascular physiology and correction of cyanosis. There should also be protection against pulmonary hypertension related to high flow through the collaterals or through a systemic shunt.

• If adequate pulmonary circulation is achieved by confluent PAs, the next step would be an elective VSD closure and RV to PA homograft conduit. The timing of such procedure differ between institutes and is largely left to the comfort of surgical team and availability of appropriate sized homograft.

• If the pulmonary arteries remain diminutive and the collaterals are small, it may be prudent to perform a central shunt to promote growth of the native pulmonary arteries at early stages. The child can then be serially evaluated to determine the status of PA growth.

• The most disappointing subgroup is the absence of native PAs with suboptimal collaterals. In this group, neither the unifocalization nor the central shunt is possible. Such children are left for the natural history, but they evoke interest in the genetic analysis.

Genetics of PA-VSD

In 1996, Digilio et al made an attempt to explain the genetics of TOF and its variants. In their study published in AJC titled ‘Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia’ they proposed a possible genetic syndrome as underlying defect.

In the same year, Momma et al in the same journal published ‘Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome’. In this article, they proposed that tetrology of Fallot with pulmonary atresia belongs to a spectrum of conotruncal cardiac malformations that are often associated with monosomy 22q11.

Matsuoka et al in 1994 in American Journal Of Medical Genetics had shown that the clinical presentation of monosomy 22q11 includes patients with conotruncal anomaly face syndrome, velo–cardio–facial syndrome and DiGeorge syndrome in the article titled ‘Confirmation that the conotruncal anomaly face syndrome is associated with a deletion within 22q11’.

Conclusion

The fascination of correct diagnosis often leads to frustration in complex pulmonary atresia, VSD and MAPCAs. Most of the effort should be invested in correct diagnosis and to ascertain the possibility of unifocalization. Whenever possible, the initial palliation should be to promote the growth of native PAs. Better understanding of genetic issues might modify the future strategies of approach to the problem.

Please forward your questions and suggestions to the comments box or to drkiranvs@gmail.com This should be an interactive section and those who can answer can post them in the same way.

With this, let us get back to our regular feature: interesting learning scenarios

THE DUCTAL PUZZLE

How many times one comes across a large PDA in TAPVC? Theoretically, it is rare. High Qp of CCHD and large PDA generally does not go together. We had a neonate with obstructed TAPVC. We did notice a large PDA on echo, but the confirmation took a side line because I could not visualize the vertical vein. The common confluence of pulmonary veins were visualised but the distal connections were not. I could not ascertain whether it was supracardiac or infracardiac variant. Since no abnormal vascular structure was found crossing the diaphragm, I presumed it to be non-infracardiac. In the mess of finding the vertical vein, the attention on the large PDA was lost. On the table, however, the surgeons did find the PDA. Since it was not mentioned to them, the surgery got tougher than expected. Thanks to the amazing analysis and skill of our surgical team, the neonate made an uneventful recovery. The lesson was on focus and on accepting what is seen than what is written in books! Now, the idea is clear. TAPVCs can have a large PDA as association!

ONE-AND-A-HALF IN TRICUSPID ATRESIA

Actually, I never thought of this possibility. Can tricuspid atresia, despite having a reasonable sized RV, go into one-and-a-half pump repair? Tricuspid atresias are always associated with single pump corrections. How can the mandatory ASD and VSDs be closed to achieve one-and-a-half repair in the absence of tricuspid valve? One of our senior surgeons was talking about this possibility and some of the case reports about this. The technique, as I can imagine, must be very different than the conventional one-and-a-half repair. Anyone has data on this? Please let us all know.

NATURAL REGURGITATION

What is the natural history of AV valve regurgitation in AV canal defects? “It is the PAH that finishes them before anyone can study the natural history of AVVR” was the retort by one of our senior surgeons. The point is well taken. However, the question is, in partial and transitional AV canal defects or in canal-TETs, is the AVVR different from that of innate AVVR in non-AV canal defects? When we talk of unbalanced AV canals which are destined for single ventricle pathways, does the AVVR increase with age or the heart gets adapted for AVVR and ends up working better over time? “AVVR begets AVVR” is an adage in general. Is it true for AV canal defects too? If anyone has the answer on the natural history of AVVR in AV canal defects of non-PAH type, please let me know.

Q WAVES OF ALCAPA

This one was another eye-opener. We came across a 2-month-old with recent history of viral infection and deterioration of general condition. Chest radiograph taken outside showed cardiomegaley. An echo done outside was reported as dilated cardiomyopathy. The parents were referred to us for second opinion. On echo at our place, I could see a tortuous RCA with normal calibre. Three small vessels erupted out of the left coronary sinus instead of one LMC. The flow across MPA was normal and I did not find any diastolic flow across the sinuses of pulmonary artery. On ECG, we found prominent Q wave in aVL and aVR. Small q waves were seen in V2. There were no reciprocal changes. Our senior consultant, Dr Sejal Shah was not happy with the disparity between the ECG and clinical picture. On repeating the echo, we found the atypical origin of LMC from main pulmonary arterial sinus. The small vessels which seemed to erupt from the LMC location of left coronary sinus were probably artefacts. The diagnosis of ALCAPA was established and the novelty of picture was educative. The question was “Can significant Q wave in a single lead in ECG be predictive of ALCAPA?” The answer I found that day was, YES.

X IN THE MIDDLE

We found one of our patients presenting with indeterminate loop of ventricles in a situs ambiguous situation. The great arteries were d-malposed, but even with best of the effort, we could not clarify the ventricular looping. The final report went as {A,X,L}! How many times do such situations happen? Please let us know your experiences with indeterminate ventricular loops.

PEDIATRIC CARDIOLOGY PEARLS

116. In Sudden Cardiac Death, the term SUDDEN has been used to describe various intervals, but usually refers to a time <1 hour from the onset of symptoms to death or irreversible neurologic injury. In distinction, sudden death is defined by the World Health Organization as “a natural death within 24 hours of the onset of symptoms” irrespective of the cause. However, if the interval between the onset of symptoms and biologic death is limited to <2 hours, a cardiovascular cause of death is identified in 88% of all cases. (Kuller L, Lilienfeld A, Fisher R. An epidemiological study of sudden and unexpected deaths in adults. In journal Medicine in the year 1967 page 341)

117. When administered as a rapid intravenous bolus, adenosine produces transient AV node block, terminating tachycardias using the AV node. (Camm AJ, Garratt CJ. Adenosine and supraventricular tachycardia. In New England Journal of Medicine in the year 1991 page 1621)

118. Historically, neural mediated syncope was classified according to the response to a tilt table test. The response to tilt table testing was classified according to isolated or combined changes of heart rate and blood pressure. Sutton's first definition referred to (i) a vasodepressor type, reflecting mainly a drop in blood pressure at the time of syncope without changes in heart rate; (ii) a cardioinhibitory type with a decrease in heart rate and/or asystole; and finally (iii) a mixed type reflecting both a decrease in heart rate and blood pressure. (Sutton R, Peterson M, Brignole M, et al. Proposed classification of tilt induced vasovagal syncope. In European Journal of Cardiac Pacing and Electrophysiology in the year 1992 page 180). A new classification has been proposed by the European Task Force on Syncope in 2001.

119. The standard ECG record consists of 12 leads recorded from nine body surface locations with the patient in the supine position. (Bailey JJ, Berson AS, Garson A Jr, et al. Recommendations for standardization of leads and of specifications for instruments in electrocardiography and vector cardiography: Report of the Committee on Electrocardiography and Cardiac Electrophysiology of the Council on Clinical Cardiology, American Heart Association. In journal Circulation in the year 1990 page 730)

120. By light microscopy, the cells of the sinus node are morphologically distinct from the surrounding atrial myocardium; they are smaller, more compact, and contained within a fibrous matrix. (Ho SY, Anderson RH. Embryology and anatomy of the normal and abnormal conduction system. In: Gillette PC, Garson A Jr, eds. Pediatric Arrhythmias: Electrophysiology and Pacing. Philadelphia: WB Saunders, published in year 1990 page 227)

That brings us to the end of another post. Please send your comments and criticism to drkiranvs@gmail.com Also, for the information of everyone interested in pediatric cardiology, NH is organising a CME on pediatric cardiology, mainly meant for paediatricians on Saturday, 29th January, 2011. It is interestingly titled “What, When and How: Anecdotal approach to pediatric cardiology”. There is no registration fee. Entry is only by a prior free registration. If anyone is interested in participating, please send an email to my mail id. I shall let you know the procedure.

Regards

Kiran

2010-11-28T23:00:00.000+05:30

This is Dr Kiran welcoming all to the new post of NH blog. I would like to know the usefulness of the segment on current evidences. I understand that it is quite dry and interspersed with so many strange-looking names and studies. But, that is how the evidence based analysis look like! Probably, this is one segment in medical practice which needs cosmetic lift-up to get better mass appeal!

This post, I am gathering evidence on the ‘Use of Sildenafil in post-op states in CHD’. This would be followed by our regular features on learning scenarios and Pearls.

Let us start with the evidence basis for ‘Use of Oral Sildenafil in Pediatric Patients with Congenital Heart Disease in post-operative setting’.

The current use of oral sildenafil in Pediatric population is largely an extrapolation of adult studies pertaining to pulmonary hypertension. However, the comparison is not that straight forward. The possible hemodynamic variants associated with congenital heart diseases are extensive. CHD may have absence of a crucial pulmonary valve, may come with serial and parallel circulations, pulmonary hypertension of high flow against high resistance, ala-carte designed surgical states to suit the anatomy and so on. So, unless the fundamentals are understood well, it may not be appropriate to use sildenafil as a knee-jerk response. Adding to the woes, there are no clearly definable outcome parameters to define the efficacy and results with treatment of sildenafil in the immediate post-op states.

Q1. Is sildenafil therapy logical in post-op CHD status?

In 1993, Celermajer et al showed that there is pre-existing pulmonary endothelial dysfunction caused by preoperative high flow pulmonary hemodynamics, which may alter the course in post-op states. In their article titled ‘Impairment of endothelium-dependent pulmonary artery relaxation in children with congenital heart disease and abnormal pulmonary hemodynamics’ in the journal Circulation, they discussed this issue extensively. The same point was proved with simian experiments by Glavind-Kristensen in 2002 for post bypass status. Their article titled ‘Pulmonary endothelial dysfunction after cardiopulmonary bypass in neonatal pigs’ was published in Acta Anaesthesiology Scandinavia. In the same year, Lindberg et al published their article ‘How common is severe pulmonary hypertension after pediatric cardiac surgery?’ in JTCS. They found that iNO therapy is frequently indicated for the stabilization of pulmonary vascular reactivity, and decreased incidence of postoperative pulmonary hypertensive crisis. However, abrupt discontinuation of iNO was a major problem. In 1996 Atz et al had written on ‘Rebound pulmonary hypertension after inhalation of nitric oxide’ in ATS. Hence, there was a need for another treatment modality to prevent rebound PHT caused by abrupt discontinuation of iNO in post-extubation period. In 1999, Jackson et al showed the ‘Effects of sildenafil citrate on human hemodynamics’ in AJC. This was followed by another article by Atz et al titled ‘Sildenafil ameliorates effects of inhaled nitric oxide withdrawal’ in the journal Anesthesiology in the same year.

Q2. Is the supposed efficacy of Sildenafil proven?

In 2005, Karatza et al published the first study of Sildenafil in children in International journal of Cardiology, titled ‘Safety and efficacy of sildenafil therapy in children with pulmonary hypertension’. Here, they compared the efficacy and pharmacokinetics of Sildenafil with adult population as control and found equal efficacy. In 2007, in an article titled ‘Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects’, Raja et al showed the efficacy of Sildenafil in the post-op CHD states by noticing a significant decrease in PA pressure compared with baseline values after starting sildenafil, without changes in CVP and systemic arterial pressure. In the same year, Peiravian et al published ‘Oral sildenafil to control pulmonary hypertension after congenital heart surgery’ in Asian Cardiovascular and Thoracic Annals. Here, they demonstrated the use of Sildenafil in post biventricular repair.

Q3. Is there a role of Sildenafil in Post-Fontan surgery?

Khambadkone et al in 2003 published ‘Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation’ in the journal Circulation. They showed that PA pulsatility is crucial for endothelium-dependent pulmonary vasodilation. However, Fontan circulation takes away the PA pulsatility. In 2008, Rossi et al showed that ‘Sildenafil improves endothelial function in patients with pulmonary Hypertension’ in an article published in Pulmonary Pharmacology and Therapeutics. This finding extrapolated that any patient with Fontan procedure is likely to benefit from Sildenafil. Apart from this, in 2008 Giardini et al showed ‘Effects of silenafil on haemodynamic response to exercise and exercise capacity in Fontan patients’ in European Heart Journal, demonstrating the improvements found in the hemodynamic profiles in post-Fontan patients on Sildenafil. Earlier in 2006, Haseyama et al in JTCS had shown ‘Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome’ where they demonstrated an improvement in the plastic bronchitis. In the same year in ATS, Uzun et al had shown ‘Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan’. However, it is well understood that in patients with Fontan procedure, improvement of tissue oxygenation and hemodynamic parameters are more important than mean PA Pressure decrement.

Overall, it can be concluded that, in the present level of evidence, Sildenafil can be used safely in postoperative pediatric patients with CHD. It appears to be effective, or at least protective, against rebound PHT caused by withdrawal of iNO.

With this, let us get back to our regular feature: interesting learning scenarios:

ACUTE SEVERE AND CHRONIC

In acutely compromised hearts, is better to accept a natural deterioration or to go on a high-risk endeavour? We had a 10-year-old with acute deterioration of a chronic MR with a very vague history. Although her LV function could be pepped up with inotropic support, her third spacing was enormous. She had bilateral massive pleural effusions, ascitis and trace pericardial effusion. Left alone, we felt she may not get any better with medical management. However, the surgical risks are multiple-fold high in such patients. The only saving grace was preserved LV function, which could give some hope to patient and surgeon! It was contemplated that the existing third spacing is likely to add on to the post-op woes. One suggestion was to use peritoneal dialysis for about 72 hours and slowly decongest the accumulations. This would presumably reduce the myocardial edema and improve contractility. However, this idea was not considered effective by the entire team. The questions are: Is the venturing into high risk surgery in such cases advisable? Should more time be spent on medical management? Is PD a choice for consideration? Please let me know your opinions.

ONE AV, TWO SEMILUNARS

Is there a situation in left heart which parallels tricuspid atresia 3C? We had an infant diagnosed as TAPVC from outside. However, on echo, we found an obstructed cardiac TAPVC with a barely visible LA cavity. The mitral valve was imperforate. The LV was smallish, fed only by a couple of muscular VSDs shunting R to L. The RA and RV were dilated. The LV gave rise to reasonable sized aorta (Z score of minus 1) and RV to well developed, dialted PA. The ascending and transverse aortae were normal sized. The baby was symptomatic largely due to obstructed TAPVC and unusually increased Qp. One of the possibilities we thought was repair of TAPVC, DKS with BT shunt. With aortic valve being reasonable, is there any other possibility? What would the natural history of VSDs in such situations? Please let me know your ideas on the management of such issues.

JUXTADUCT

This one has always remained elusive. I don’t know if I have discussed this issue earlier. It becomes very difficult to assess the status of coarctation in the presence of patent arterial duct. We had a 4-year-old who had undergone a balloon coarctoplasty in an outside hospital in her infancy. She was being followed up. When she came to us, we found a moderately restricted PDA with a narrow juxtaductal segment. There was only systolic gradient across the narrow segment with no diastolic spillage. The classical teaching is that the presence of duct interferes with evaluation of coarctation. In this instance, we are not sure of the significance of coarctation. There were no systemic clinical signs of CoA. We contemplated the unmasking of CoA once the duct is closed. The CT may help us in this issue by a 3D reconstruction. Even if there is no CoA on CT at baseline, would the large aortic disc of PDA device compromise the diameter of juxtaductal segment further, thereby creating a CoA? I felt it is better to subject the patient for surgical ligation of PDA. We can always check for the CoA after PDA ligation on table and take a call. What is the advice of readership? How would you like to tackle this case? Please let all know.

DILATED PA

We recently had a 25-year-old referred for abnormal Chest radiograph from outside. She was otherwise asymptomatic. On examination, there was a click in the pulmonary area. Echo showed doming pulmonary valve leaflets with no gradient across. The MPA was significantly dilated. The estimated PA pressures were normal. How far are these pictures common? How frequently should such patients undergo repeat evaluations and what to look for? Is there a management plan for them? Is the supposed progress preventable by medications? There appears to be lot of data on bicuspid aortic valves. However, no such data seems to exist for this category. If anyone has any such instances in their follow up, please let us know.

SEPTOSTOMY Vs BTT SHUNT

This issue probably needs much more discussion in the professional circles. I have brought up this issue in the previous posts too. When it comes to mixing lesions, the capacitance chambers are much superior to contracting ones. That is the reason why a moderate sized ASD proves to be a superior option than a large VSD in TGAs. When a TGA with restrictive ASD gets desaturated, adding BTT shunt does no good. In fact, it may worsen the scenario. Extending the logic, this should be true for any d-malposed great artery situations, unless it is DILV! I came across one 11-year-old with 100% DORV saturating 56%. The PS was mild and the PAs were dilated. There was a subarterial VSD of about 40% of aortic annulus with a PFO. In this case, even the best of streaming cannot deliver a good amount of oxygenated blood into Aorta. How different the scenario is if there were to be a non-restrictive ASD? Please let me know your opinions on this.

PEDIATRIC CARDIOLOGY PEARLS

111. Neonates are more resilient to metabolic or ischemic injury. In fact, the neonate may be particularly capable of coping with some forms of stress. Tolerance of hypoxia in the neonate is characteristic of many species, and the plasticity of the neurologic system in the newborn is well described. (Lenn NJ. Plasticity and responses of the immature nervous system to injury. In journal Seminars in Perinatology in the year 1987 page 117)

112. The physiology of the PDA is secondary to overperfusion of the lung and possibly underperfusion of the systemic circulation. Infants' lungs are fully recruited at rest so that any increase in flow from left-to-right shunts predictably increases fluid filtration in the lung. (Alpan G, Scheerer R, Bland R, et al. Patent ductus arteriosus increases lung fluid filtration in preterm lambs. In journal Pediatric Research in the year 1991 page 616)

113. Elevated transpulmonary pressure gradient and elevated pulmonary vascular resistance have been identified as risk factors for early mortality after heart transplantation. (Kirklin JK, Naftel DC, Kirklin JW, et al. Pulmonary vascular resistance and the risk of heart transplantation. In Journal of Heart Transplantation in the year 1988 page 331)

114. Catheter therapy has now been applied to the cure or modification of most pediatric arrhythmias, including AV node re-entry tachycardia, ectopic atrial tachycardia, atrial re-entry/flutter, congenital junctional ectopic tachycardia, and some forms of ventricular tachycardia. (Kugler JD, Danford DA, Deal BJ, et al. Radiofrequency catheter ablation for tachyarrhythmias in children and adolescents. The Pediatric Electrophysiology Society. In New England Journal of Medicine in the year 1994 page 1481)

115. A release of nickel from the device with a peak at 1 month postimplantation has been described. However, its clinical significance is questionable, and reports of clinically significant allergic reactions to nickel after device implantation are rare. (Lai DW, Saver JL, Araujo JA, et al. Pericarditis associated with nickel hypersensitivity to the Amplatzer occluder device: A case report. In journal Catheterization and Cardiovascular Interventions in the year 2005 page 424)

This brings us to the end of another post. Please send your comments and suggestions to drkiranvs@gmail.com Hoping to add some features on to the existing segments. Please suggest if any novel ideas occur!

Regards

Kiran

2010-11-17T23:33:00.000+05:30

Dr Kiran welcomes all to the new post of NH blog. As previous, we shall see a review on the current controversies, few learning scenarios and the pearls. Thanks for the feedback on the section on controversies and hope to continue. Please suggest topics of interest from your side too.

In this post, we will see the current evidence on the use of antiplatelet agents in pediatric practice with special emphasis on cardiac patients.

Q1. What are the currently available antiplatelet agents for pediatric use?

1. Aspirin: inhibits platelet function by irreversibly binding to cyclooxygenase (COX)-1
2. Dipyridamole: inhibits adenosine uptake into platelets
3. Clopidogrel: irreversibly binds to the purinergic P2Y12 receptor on platelets
4. Intravenous GP 2b/3a inhibitors abciximab, eptifibatide, and tirofiban: bind to platelet GP 2b/3a receptors, which prevents their cross-linking with fibrinogen and blocks the last common pathway of platelet aggregation

Q2: Why is antiplatelet therapy for children different from adults?

Two separate studies by Rajasekhar and collegues discussed the differences in the functional activities of platelets in newborns and that from adult population. The first was during 1994, in an article titled ‘Neonatal platelets are less reactive than adult platelets to physiological agonists in whole blood’ in the journal Thrombosis and Haemostasis. This was followed by an article in the same journal in 1997 titled ‘Platelet hyporeactivity in very-low-birth-weight neonates’. Israles and colleagues published their data in 2001 in the Journal of Pediatrics. The article was titled ‘Evaluation of primary hemostasis in neonates with a new in vitro platelet function analyzer’ which showed a decreased platelet functional response in neonates than adults. These findings suggested that neonates may require lower weight-adjusted doses of antiplatelet agents to achieve the same level of platelet inhibition as for adults and older children.
Apart from the physiological differences, some practical problems are also observed. The children are more mobile and more likely to fall and injure themselves. This is added on with their lesser degree of understanding and adherence to instructions. Reye’s syndrome is a potential complication. Also, they are less likely to understand the consequences of stopping the therapy or not adhering to the dosage.

Q3: What are the present cardiac interventional indications for antiplatelet therapy in children?

The present cardiac indications would be children who undergo implantation of cardiac devices (such as ventricular assist devices (VADs), septal defect occluders, and stents)
An expert review was published in 2008 by Fu and Hijazi under the title ‘The Amplatzer Septal Occluder, a transcatheter device for atrial septal defect closure’ where they strongly advocated the use of aspirin post-ASD device closure. They have quoted the famous study by Fischer et al published in journal Heart in 2003 with title ‘Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder: a single centre study in 236 consecutive patients’. In this study, aspirin was used in all 236 and just one patient had a neurological event after 4 years, attributable to device. The evidence is thus in favour of aspirin in this setting.
The data on aspirin with stent placement is not enough, largely due to lesser numbers done in pediatric population. In a retrospective review published by Ebeid et al in 1997, of nine patients (four were 18 years old or younger), aspirin therapy was administered for at least 1 year after implantation of balloon-expandable stents for coarctation of the aorta, and no patient showed evidence of thromboembolism after an average follow-up period of 18 months. This was published in JACC under the title ‘Use of balloon expandable stents for coarctation of the aorta: initial results and intermediate-term follow-up’. Kutty and Zahn published their data on use of aspirin after stent placement in pediatric population in 2008 in the journal Catheter and Cardiovascular Interventions under the title ‘Interventional therapy for neonates with critical congenital heart disease’. In the same year, Menon and colleagues published ‘Hybrid intraoperative pulmonary artery stent placement for congenital heart disease’ in the AJC. In this retrospective review, 24 patients (14 of whom younger than 18 years) received aspirin after undergoing hybrid pulmonary artery stent implantation secondary to congenital heart disease and did not demonstrate any episodes of stent thrombosis over a mean follow-up period of 19 months.

Q4: What are the cardiac post-surgical indications for antiplatelet therapy?

Any child who needs a systemic-to-pulmonary artery shunt is at the risk of thrombotic event. The studies quoting uselessness of therapy were very small and underpowered (Al Jubair et al in 1998 in Journal Cardiology in the Young ‘Results of 546 Blalock-Taussig shunts performed in 478 patients, Fenton et al in ATS in 2003 ‘Interim mortality in infants with systemic-to-pulmonary artery shunts’ and so on) the overall data is in favour of using aspirin (Motz et al in 1999 in journal Cardiology in the Young ‘Reduced frequency of occlusion of aortopulmonary shunts in infants receiving aspirin’, Li et al in 2007 in journal Circulation ‘Clinical outcomes of palliative surgery including a systemic-to-pulmonary artery shunt in infants with cyanotic congenital heart disease: Does aspirin make a difference? and so on).
In 2002, Monagle and Karl published their data as ‘Thromboembolic problems after the Fontan operation’ in Seminars in Thoracic and Cardiovascular Surgery. They advocated the use of aspirin, as children a with single-ventricle approach the Fontan procedure, they remain at high risk of thromboembolic complications.
The data on the use of antiplatelet agents and anticoagulation agents in post-Fontan group remains conflicting. Seipelt and co-workers wrote ‘Thromboembolic complications after Fontan procedures: comparison of different therapeutic approaches’ in ATS in 2002. This was a retrospective study investigating 87 survivors of Fontan surgery, the rates of thromboembolic complications were similar for the patients who received aspirin (1.6 events/100 patient-years) and those who received warfarin (1.1 events/100 patient-years) but much higher for those who received no antithrombotic therapy (4.2 events/100 patient-years). The journal Circulation published an abstract by Monagle et al in 2008 presented at AHA meeting. It was a multicentre randomized trial comparing heparin/warfarin versus aspirin as primary thromboprophylaxis for two years after Fontan procedure in 111 children with mean age of 3.8 years. During a 27-month follow-up period, thrombosis occurred for 24% of the heparin and warfarin recipients and 21% of the aspirin recipients (p = 0.47). The rate of major bleeding also was the same (1 event per group). These data suggest that anticoagulation and antiplatelet therapies are similarly safe and effective in preventing thrombosis after Fontan surgery. However, the favour of surgeon still remains with warfarin in large many institutes across the globe.

Q5: Role of clopidogrel

The year 2005 saw a review article by Finkelstein and colleagues on ‘Clopidogreal use in Children’ in the Journal of Pediatrics. This review served as the baseline for further research with clopidogrel. In 2008, Maltz et al published ‘Clopidogrel in a pediatric population: safety and efficacy results from a single center’ in journal Pediatric Cardiology. It was a retrospective analysis of 90 children (median age, 6.7 years) receiving various doses of clopidogrel. It had a favourable safety and tolerability profile because only two children, both of whom also received aspirin, experienced major bleeding. An additional three patients receiving aspirin plus clopidogrel experienced minor bleeding.
The only randomized control study on Clopidogrel hitherto has been published by Li et al in 2008 in journal Circulation. Popularly called the PICOLO trail, the article is titled ‘Dosing of clopidogrel for platelet inhibition in infants and young children: primary results of the Platelet Inhibition in Children On cLOpidogrel trail’. In this, 92 young children aged 0 to 24 months with cardiac disease were randomized to receive placebo or clopidogrel 0.01, 0.10, 0.15, or 0.20 mg/kg/day. Via platelet aggregometry, clopidogrel 0.20 mg/kg/day was identified as the dose that provided a level of platelet inhibition similar to that achieved with 75 mg/day for adults. Notably, none of the patients experienced serious hemorrhagic events, and only four patients (2 in the placebo group and 1 each in the clopidogrel 0.01 and 0.20 mg/kg/day groups) experienced minor bleeding. The results of PICOLO underscore the importance of conducting randomized clinical trials with children, particularly dose-finding studies, because the 0.20-mg/kg/day clopidogrel dose identified in PICOLO is five times lower than the 1-mg/kg/day dose extrapolated from adult studies.
The ongoing CLARINET study may come out with the answers sought after by the pediatric cardiologists. The details of the trail can be accessed at http://clinicaltrials.gov/ct2/show/NCT00396877 The trail is by Sanofi-Aventis and Bristol-Myers Squibb and titled ‘Efficacy and safety of clopidogrel in neonates/infants with systemic to pulmonary artery shunt palliation (CLARINET)’ and is using clopidogrel at a dose of 0.20mg/kg/day.

Q6: What are the current recommendations for the use of antithrombotic therapy in children?

The current guidelines are drawn from the 2008 report by Monagle and others published in journal Chest. It is the 8th edition of American College of Chest Physicians Evidence-Based Clinical Practice Guidelines on Antithrombotic therapy in neonates and children. The following are excerpts from the content:
• For children with mechanical prosthetic valves who experience thrombotic events while receiving Vit K Antagonist therapy or who cannot tolerate Vit K Antagonist therapy, ASA should be used (Grade 2C)
• Antiplatelet therapy, either ASA (1–5 mg/kg/day) or Dipyridamole (3–10 mg/kg/day), should be instituted within 72 h of Ventricular Assist Device placement (Grade 2C)
• For children who have a Modified BTT shunt, postoperative ASA (1–5 mg/kg/day) or no antithrombotic therapy should be used compared with prolonged therapy using Low Molecular Wt Heparin or Vit K Antagonist (Grade 2C)
• For children who undergo the Norwood procedure, Ultra-Fractionate Heparin immediately after the procedure, with or without ongoing antiplatelet therapy, should be used (Grade 2C)
• For children who undergo Fontan surgery, ASA (1–5 mg/kg/day) or therapeutic Ultra-Fractionate Heparin followed by Vit K Antagonists should be used (Grade 1B)
• In the absence of a documented ongoing cardioembolic source, no anticoagulation or ASA therapy should be given to neonates with first AIS (Grade 1B)
• For neonates with recurrent AIS, anticoagulant or aspirin therapy may be given (Grade 2C)
• For children with AIS not secondary to sickle-cell disease, cardioembolism, or dissection, Ultra-Fractionate Heparin, Low Molecular Wt Heparin, and ASA (1–5 mg/kg/day) are all acceptable options that should be used for 2 years or longer (Grade 1B)
• Children who experience recurrent Acute Ischemic Stroke or Transient Ischemic Attack while taking ASA should be switched to either clopidogrel or anticoagulant therapy (Vit K Antagonist or Low Molecular Wt Heparin) (Grade 2C)
• For children with Kawasaki disease, high-dose ASA (80–100 mg/kg/day) should be given as an anti-inflammatory agent for up to 14 days, followed by low-dose ASA (1–5 mg/kg/day) for 6–8 weeks as prevention against Coronary Artery Aneurysm (Grade 1B)

That brings us to the end of this discussion. The evidences are still pending. However, I have tried to put available stuff for the current decision making. It can help the decision makes to avoid idiosyncrasy and to instill a uniform policy in the work-front. Also, the junior staff may find it useful to clear their own understanding of the current status. Any recommendations on this topic or any suggestions to improve this section can please be sent to drkiranvs@gmail.com The suggestions would be placed in the subsequent posts, if they come and if the posts continue!!

With this, let us get back to our regular feature: Interesting learning scenarios

FIRST OR SECOND

It is well understood that the Tricuspid atresia with NRGA outnumber the other anatomical variants. Most of the children would eventually complete their single ventricle pathways belong to Tricuspid atresia Ib. Is there any study on the number of Tri atrsia IIb having gone through the Fontan completion? Theoretically, Ib can have two sequential restrictions to Qp: one at VSD and other at pulmonary valve. Whereas, for the IIb variant, this reduces to only the latter. Can this contribute to the eventual numbers? Off late, many of the IIb variants I have come across could not make it to the operating table for having not met the criteria. It would be interesting to note the data from various other centres. If anyone have thought of this question, please let me know your observations.

BLUE IS BETTER

This was a paradoxical situation. When the ventricular EDPs are high in a congenital heart disease, the surgical and ITU teams have to exercise extra-caution. We had a 44-year-old with tetralogy-absent pulmonary valve disease with NYHA class III symptoms and baseline SO2 of 89% in room air. Her LV EDP was 22mmHg. The question was on the expectations from surgery in this scenario. The surgical team was not convinced that the surgery would relieve her symptoms, unless a pulmonary component is co-existing. However, the cardiologist was arguing that the high LV EDP is likely to be the culprit and surgery would be beneficial. Our senior surgeon brought out a point: The EDP of a cyanotic ventricle would improve with surgery and that of acyanotic would not. In other words, in adult TOF, the surgery would reduce the high EDP, but not in VSD. The argument sounded logical. Are there any studies or observations in this regard? Please let me know your take on this.

THE SEPTECTOMY SAGA

I came across a 2-month-old with large VSD with pulmonary atresia. This baby also had a hypoplastic TV, hypoplatic muscle bound RV and a stretched PFO with flap open towards LA, shunting Right to Left. The PAs were small sized and the baby saturated 70% in room air. Ideally, there is a need for increasing the Qp and advocating BTT shunt appears logical choice. However, with increase in LA return, would the PFO flap close towards RA? Once this happens, where would the RA decompress? The TV and RV appeared too small to negotiate the RA outflow. Would it not be detrimental to do the BTT shunt alone? Should we be creating a simultaneous ASD? The recommendation was to do an atrial septectomy clubbed with BTT shunt. However, for same reasons discussed in previous posts, this was not perceived as a good idea. Is there an alternate logic? Am I missing something that the surgeons are confident of? What is the experience of the readership in this issue? Please let me know. I shall try to keep the progress of this baby updated in the subsequent posts.

TYPING AND SIZE

What is the criterion for the Rastelli classification of AV canal defect? The individual opinions vary, but it remains the nature of AV valve itself. We had a newborn with complete AV canal defect. The bridging leaflet was sending its chordae onto the crest of IVS. However, the left AV valve component was hypoplastic, resulting in hypoplastic LV. This was followed by DORV and membranous pulmonary atresia. The management would be on single ventricle lines. However, there was some ambiguity on classifying this as type A Rastelli Vs type C. What is the vote of readership? Please let me know how you classify the AV canal defects in your routine practice.

EVIDENCE OF COMPROMISE

We often predict the loss of ventricular cavity in tunnel closures of VSD, especially in DORV, d-malposed setting. However, the assessment is on 2D and that too for RV, which is not a very echo-friendly structure! When we actually, predict such a scenario, are talking of the volumes or functional cavity or total area of contractile elements? Which of them would be an ideal measurement? Depending on the answer, we can actually devise our investigation in future. If anyone has data on this, please inform me.

PEDIATRIC CARDIOLOGY PEARLS

106. There are two frequent variants of the left aortic arch. One is common brachiocephalic trunk, in which the right innominate and left carotid arteries arise from a single origin. This is present in 10% of otherwise normal left arches (29) and usually is of no consequence. The other variant is separate origin of the left vertebral artery from the aortic arch proximal to the takeoff of the left subclavian artery rather than from the subclavian artery. This too is seen in 10% of normal left arches. (Edwards JE. An atlas of Acquired Diseases of the Heart and Great Vessel. Published by WB Saunders, Philadelphia in the year 1961)

107. Small VSDs, sometimes referred to as Roger's defect, are those less than one third the size of the aortic root and impose a high resistance to flow with a resultant large systolic pressure difference between the two ventricles. There is a small left-to-right shunt, normal right ventricular systolic pressure, and essentially normal work characteristics of the ventricles. The magnitude of the left-to-right shunt is related directly to the size of the defect, and there is no tendency for an increase in pulmonary vascular resistance. The pressure gradient across the defect favors the left ventricle throughout the cardiac cycle and can result in a continuous left-to-right shunt. (Levin AE, Spach MS, Canent RV Jr, et al. Ventricular pressure-flow dynamics in ventricular septal defect. In the journal Circulation in 1967 page 430)

108. The fundamental problem in AV canal defect is failure of the endocardial cushions to fuse, creating a defect in the atrioventricular septum. The primum atrial septal component of this defect is usually large. This results in downward displacement of the anterior mitral leaflet to the level of the septal tricuspid leaflet. (Gutgesell HP, Huhta JC. Cardiac septation in atrioventricular canal defect. In Journal of American College of Cardiology in the year 1986 page 1421)

109. Researchers have found a missense mutation in myosin heavy chain 6 (on chromosome 14q12) has been found to cause familial ASD. (Ching YH, Ghosh TK, Cross SJ, et al. Mutation in myosin heavy chain 6 causes atrial septal defect. In journal Nature Genetics in the year 2005 page 423)

110. The first application of a fluid dynamics principle in medicine was given by Roos. It was represented as “Increasing viscosity or decreasing vessel radius leads to an elevation of both arterial pressure and vascular resistance” (Roos A. Poiseuille's law and its limitations in vascular systems. In the journal Med Thoracics in the year 1962 page 224)

That brings us to the end of another post. Please send your responses and reactions to drkiranvs@gmail.com or use the comments box. Please keep the discussions open. I am trying for an interactive module for debating in pediatric cardiology. Please send in your suggestion on this.

Regards

Kiran

2010-11-12T19:04:00.000+05:30

Dr Kiran welcomes all to the new post of NH blog.

I should thank the responses I got for a small (?emotional) outburst in the last post. My teachers whom I revere a lot, my friends who have a cordial relationship, my colleagues who I thought would never have read the blog – all came together to assure that the blog is being read and for various reasons it should be continued. That was indeed a great reinforcement.

There was a suggestion also: that I should invite few big people to write for the blog. To the best of my knowledge, I do it in every post! Of course, neither the name nor the pattern of blog suggests that I own the blog. Let me reiterate once again: The blog belongs to everyone who holds any passion towards its objective put up below the masthead. All are formally invited to be a part of the content of this blog once again.

The section of “Current controversies in Pediatric Cardiology” seems to have evoked better response. I shall try to continue this section till I get drained off the ideas!

This time in this segment, I am dealing with the use of cath data in decision making. This is possibly one area which has minimal consensus and maximal idiosyncratic interpretation. We shall see the available evidence for this.

Q1: Which anesthetic agents should be used for cath studies in children?

Various drug protocols are drawn for this purpose, but the supporting literature is not uniform. Laird et al in 2002 presented their data in the journal Anesthesia and Analgesia under the title ‘Pulmonary-to-systemic blood flow ratio effects of Sevoflurane, Isoflurane, Halothane, and Fentanyl/Midazolam with 100% oxygen in children with congenital heart disease’, in which they discouraged the use of inhaled drugs for their myocardial depressant effects. Of the intravenous choices, long back in 1990, Berman et al in their article titled ‘Hemodynamic effects of Ketamine in children undergoing cardiac catheterization’ published in journal Pediatric Cardilogy had observed that Ketamine given in boluses would increase pulmonary vascular resistance, raking up a controversy. In a study published by Williams et al in 1999 in the journal Anesthesia and Analgesia titled ‘The hemodynamic effects of Propofol in children with congenital heart disease’, it was noted that Propofol given as a single drug seems to decrease systemic vascular resistance, making calculations of shunts unreliable. The same opinion was shared by Oklu and colleagues in 2003 in their article ‘Which anesthetic agent alters the hemodynamic status during pediatric catheterization? Comparison of Propofol versus Ketamine’ published in the Journal of Cardiothoracic and Vascular Anesthesia. In this prospective randomized study, continuously administered ketamine did not affect pulmonary vascular resistance significantly. Few workers in this field decided to go for low dose combination modules. Jobier et al used Midazolam with Ketamine. Their data was published in journal Pediatric Cardiology in 2003 under the title ‘Use of low-dose Ketamine and/or Midazolam for pediatric cardiac catheterization: is an anesthesiologist needed?’ In the same year, Kogan et al published ‘Propofol-Ketamine mixture for anesthesia in pediatric patients undergoing cardiac catheterization’ in the Journal of Cardiothoracic and Vascular Anesthesia. These two studies have formed the basis for the evidence in this regard, under class IIa indication.
In pediatric patients undergoing cardiac catheterization for hemodynamic measurements, sedation and analgesia can be achieved by combining midazolan with fentanyl, or by using low-dose ketamine in association with either midazolan or low-dose propofol. Continuous intravenous administration is preferable. Multi-drug cocktails, inhaled agents, propofol as single drug and bolus administration of any drugs should be avoided. (Level of evidence: B)

Q2. How to assess and interpret Qp, Qs, SVRI and PVRI?

Presumed entities while using Ficks principle have jeopardized the calculations since its onset! Yet, it is continued for the lack of better alternatives. The landmark article by Wilkinson in the journal Heart in 2001 titled ‘Congenital heart disease: haemodynamic calculations in the catheter laboratory’ serves as the basic manual for formulae and technical advice. As per the advice from Wilkinson, if an assumption is considered, then more than one value should be obtained. The classical paper of LaFarge and Miettinen on ‘The estimation of oxygen consumption’ published in journal Cardiovascular Research in the year 1970 serves as the core guide and has universal usage. However, it should be acknowledged that in infants and toddlers, it provides higher oxygen consumption values making the calculations appear ridiculous sometimes. To overcome this issue, two studies have come up. In 1989, Lindahl wrote ‘Oxygen consumption and carbon dioxide elimination in infants and children during anaesthesia and surgery’ in British Journal of Anaesthesia. This was followed by ‘Oxygen consumption in infants and children during heart catheterization’ by Lundell and co-workers in the journal Pediatric Cardiology in 1996. These values settled the issue for some extent but are still far from accurate. The problem intensifies in cyanotic children especially for Fontan circulation. Shanahan et al in 2003 published ‘The influence of measured versus assumed uptake of oxygen in assessing pulmonary vascular resistance in patients with a bidirectional Glenn anastomosis’ in journal Cardiology in the Young. Here, they noted that the measured values of oxygen consumption were far greater than the assumed ones and suitability for Fontan completion may not be reliable with the data obtained. Bergstra et al had published ‘Assumed oxygen consumption based on calculation from dye dilution cardiac output: an improved formula’ in the European Heart Journal in 1995. The same was seconded in 2000 by Berger in European Heart Journal again in article ‘Possibilities and impossibilities in the evaluation of pulmonary vascular disease in congenital heart defects’. Dye dilution can be a suitable bridge before something better comes up. Hence the recommendation:
In patients with congenital heart disease associated with pulmonary hypertension, pulmonary and systemic blood flow and vascular resistance should be calculated preferably using measured oxygen consumption, with attention to all other potential sources of error. (Indication class: IIa, level of evidence: B)
If measured values of oxygen consumption cannot be obtained, calculations should be performed assuming alternative values at the upper and lower limits of the likely range for that given patient, and results expressed as intervals. If the likely range is too wide and a narrow one is required for decision-making, assumed values should not be used at all. (Indication class: IIb, level of evidence: B)

This sounds more logical than the words ‘cath has its own fallacies’ after making the patient and the performer go through the gruesome ordeal!

Q3: Pulmonary vasoreactivity and operability

Historically, the operability assessment was a radical step towards safety of surgery. One among the strong criticisms faced by the present technique is that at high oxygen concentrations, determination of arterial PO2 is necessary and measurement of oxygen consumption is not possible. In 1996, Berner et al published ‘Inhaled nitric oxide to test the vasodilator capacity of the pulmonary vascular bed in children with long-standing pulmonary hypertension and congenital heart disease’ in the American Journal of Cardiology. This was followed by article by Atz et al in 1999 in JACC, titled ‘Combined effects of nitric oxide and oxygen during acute pulmonary vasodilator testing’. These articles accounted pulmonary vascular resistance and pulmonary to systemic resistance ratio, and the way they change during an acute vasodilator challenge have been taken into consideration. The article by Atz also defined maximal achievable stimulation without any toxic side-effects by 10 minute administration of a mixture containing >90% oxygen and 80 ppm nitric oxide. With these values as baseline, many workers tried to improvise the effect. Cannon et al published their study in 2005 in journal Pediatric Cardiology under the title ‘Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response’ in which they used 20ppm NO without enhancing the oxygen concentration. Alternative strategy of starting at low concentrations with gradual increases until response is also advocated, but would involve longer hours and multiple samplings, both of which are not advocated in children.
Hence, the recommendations would be:
In patients with congenital heart disease and pulmonary hypertension, there are no simple ways to predict outcomes following correction of the cardiac defects. Therefore, operability (which does not imply a warranty of favourable outcome without any residual elevation of pulmonary pressures) should be defined on an individual basis, taking into account a substantial amount of clinical data as well as information derived from noninvasive and sometimes invasive evaluation. (Class I indication, Level of evidence:C)
Also, in patients with biventricular circulations and baseline elevation of pulmonary vascular resistance > 6 Wood units∙m2 and a pulmonary to systemic resistance ratio of >0.3, a >20% decrease in both parameters during inhalation of low concentrations of nitric oxide, with respective final values of <6 Wood units∙m2 and < 0.3 indicates that vasoconstriction plays a role, and suggests that from the hemodynamic point of view, correction of the defect may be considered as a reasonable approach. (Class I indication, Level of evidence:B)

This marks the end of this topic. I have taken up 3 relevant questions and drawn 5 recommendations as per the available evidence. Please let me know if you would like to have a complete bibliographic notation of the studies mentioned in the discussion. If so, I shall provide them at the end of discussion to avoid the incoherence of flow. Please send your comments and suggestions to drkiranvs@gmail.com to enable others to understand your ideas and flow of thoughts.

With this, let us continue towards our regular feature: Interesting learning scenarios.

HYPOPLASTIC LV, HYPER-CORONARIES!

This 9-day-old baby presented to us in a perfectly asymptomatic condition. On echo, we found an aneurysmal IAS with a moderate L to R shunt, small MV (Z-score minus 5), small, non-apex reaching LV with intact IVS, small aortic valve (Z-score minus 3) with near absence of antegrade flow. The hypoplastic LV was decompressing by a moderate MR. Great artery relationship was normal. What surprised us were a normal sized ascending and transverse aortae and the neck vessels. Further distally, there was a small, bidirectionally shunting PDA with not much retrograde filling. How is the brain fed? What can explain the size of proximal aorta? The surprise element of the picture was unusually dilated coronaries filling retrograde. The RCA was massive and coursed along the right AV groove. The LMC was enlarged with normal branching, but could not be traced much. The distal end of any of the coronaries could not be traced on echo. It was clear on colour Doppler that the flow in the coronaries was towards the aorta. Surprisingly, there was no wall motion abnormality or functional impairment of the right ventricle. It is the first time that I had seen such a combination. Is this described? What are the management options in this kid? Would it still require the first stage of Norwood despite having a good sized proximal aorta? Can we create a VSD to decompress the LV so as to minimise the MR? Please let me know your experiences with such scenarios. I shall keep the readership updated if I get any further info on this.

ISOLATED TR

We do come across good number of isolated sever TR in many children. They are usually not much symptomatic. The tolerability of the heart problem is good in them. The symptoms are more often non-cardiac than the other way. How should such children be treated? The ECS guidelines of 2007 specify wait-and-watch policy. Should we actually wait for RV to fail? Isn’t the prognosis worse after RV failure? Can we go by any additional data like MRI for RV volumes? Is this data good for taking a radical decision? Our senior surgeons always maintain that the patient should ‘earn’ his surgery. How would such subgroup earn the surgery? The overall data that I could gather justify the stand taken by ECS. Is the policy any different in other places? Please let me know your takes on this issue.

SEQUENTIAL OBSTRUCTION

Please take a look on this scenario. A toddler with Tricuspid atresia 1B. The VSD is reported as restrictive. Pulmonary valve is good sized and PA sizes are OK. The ASD is restrictive, as evidenced by data on echo and cath. There are no systemic symptoms like edema or pulsatile liver. If the reverse pulmonary venous wedge pressure turns out to be 16mmHg, can we confidently do a BD Glenn and an atrial septectomy? In other words, when there is a sequential obstruction (as restrictive ASD and restrictive looking VSD in this case), can we relieve the proximal (by atrial septectomy) and be confident of the status of distal (VSD)? What would happen to PA pressures with atrial septectomy? How controlled can a concomitant PA band be? When Fontan completion does appear to be on cards, can one take the risk of closing the antegrade flow via PA? These were some of the questions that came up during one of our discussions. If anyone has sorted out this scenario earlier, please let us know the possible alternatives in such cases.

ROLE OF NUMBERS

What is the yield of cath data in ASD with RV dysfunction? We came across a 13-year-old with this picture. He had a large ASD with severe PAH and RV dysfunction. He went through the cath procedure. The PA pressures were half-systemic. The RV EDP was 12mmHg. The RV injection is not done in such cases, so cath assessment of RV function was not possible. The software for calculating the RV volumes is not available with us. There were no additional lesions. There is a clear text-book indication for cath in this scenario. But, what is the yield? What cath data would have influenced a different strategy? If not a complete closure, we would have done a reduction of ASD size, leaving a fossa ovalis defect and contemplating a device closure in future as per need. Is the risk/benefit ratio justified in such scenarios? Should we be drawing a different strategy for such cases? Is my analogy wrong or am I missing something in the entire picture? Please let me know.

BATSON’S PLEXUS

Often, we find the surgical team talking of Batson’s plexus during description of AP collaterals. I found this data on vertebral venous plexus otherwise called Batson’s plexus: Any of four interconnected venous networks surrounding the vertebral column; anterior external vertebral venous plexus, the small system around the vertebral bodies; posterior external vertebral venous plexus, the extensive system around the vertebral processes; anterior internal vertebral venous plexus, the system running the length of the vertebral canal anterior to the dura; posterior internal vertebral venous plexus, the system running the length of the vertebral canal posterior to the dura; the latter two constitute the epidural venous plexus.
Throughout this description, I could not find a single link that can connect Batson’s plexus to AP collaterals of cardiovascular utility. Please let me know if any study has been conducted to show that they are indeed Batson’s plexus.

PEDIATRIC CARDIOLOGY PEARLS

101. When the degree of valvular pulmonary stenosis is severe enough to cause a decrease in fetal right ventricular output, a larger-than-normal atrial right-to-left shunt is established in utero. This condition has been termed critical pulmonary stenosis. (Freed MD, Rosenthal AR, Bernhard WF, et al. Critical pulmonary stenosis with diminutive right ventricle in neonates. In journal Circulation in the year 1973 page 875)

102. Unlike the patient with a duct-dependent pulmonary circulation and pulmonary atresia with ventricular septal defect, the caliber of the pulmonary arteries in patients with pulmonary atresia and intact ventricular septum rarely is a major determinant of outcome. It is important to recognize that significant coarctation of the left pulmonary artery associated with the site of ductal insertion can occur in the neonate and the young infant. This may be, in part, mediated by the observation that the arterial duct tends to close earlier in patients with pulmonary atresia and intact septum than it does in patients with pulmonary atresia and ventricular septal defect. (Marino B, Guccione P, Carotti A, et al. Ductus arteriosus in pulmonary atresia with and without ventricular septal defect. Anatomic and functional differences. In Scandinavian Journal of Thoracic and Cardiovascular Surgery in year 1992 page 93)

103. In Pulmonary Atresia with VSD, the blood supply to the lungs is entirely from the systemic arterial circulation. The sources are the ductus arteriosus, systemic-to-pulmonary collateral arteries, occasionally a coronary artery, and plexuses of bronchial or pleural arteries. Ductal and collateral sources may coexist in the same patient but only rarely coexist in the same lung. (Liao PK, Edwards WD, Julsrud PR, et al. Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect. In Journal of American College of Cardiology in the year 1985 page 1343)

104. Maternal treatment with trimethadione or paramethadione during the pregnancy has been associated with the development of multiple anomalies, including cardiac septal defects and TOF. (Feldman GL, Weaver DD, Lovrien EW. The fetal trimethadione syndrome. In American Journal of Diseases in Children in the year 1977 page 1389)

105. Truncal valve stenosis, when present, usually is associated with nodular and dysplastic cusps. (Butto F, Lucas R, Edwards J. Persistent truncus arteriosus: Pathologic anatomy in 54 cases. In the journal Pediatric Cardiology in the year 1986 page 95)

With this, I conclude this post. Thanks again for the support. If you come across any controversial topic that needs discussion and evidence search, please let me know. I shall try to discuss the topic, crediting you for the copyright of discussion! Please feel free to put your opinions in the comments box or use my email id drkiranvs@gmail.com to send your suggestions and comments

Regards

Kiran

2010-11-05T22:50:00.002+05:30

Dr Kiran welcomes all to this new post of pediatric cardiology blog.

I have often thought if I should wind up the blog. It takes lots of time for me to get the things compiled. It takes keen observation and note-making to get the facts correct. It takes good amount of thinking to write in a language different from my mother-tongue. When the blog was started, the name was chosen carefully so that everyone in the department would think it is their own and would contribute. All these expectations went the same way my other expectations go.... right into the drain!

Still, I am continuing it. Since the comments I get are minimum, I don’t even know if anyone is reading the blog at all! So, even if I stop, I don’t think anyone would be affected. “We never knew you existed; anyways – good bye” would probably be the response. When something does not make any difference, why bother?

Despite the apparent pessimism, I shall continue this blog as long as feasible. It gives me an opportunity to be alert, express, think and explore. It has given me new ideas on learning. It has taught me how to work (for blog) without expectation (of comments). The time investment done on this blog is not a waste for me as of now atleast. Of course, bitter pills would not matter if one is sure of the commitment.

Let me congratulate our fellows for their enthusiasm to explore. An idea originated when I was writing for one of the posts for this blog. I proposed debating for the fellows in the department as a part of their academics. I hand-picked about 25 topics and ran through the available literature. I chose those topics which had good number of pros and cons. A list of 5 topics were given to the fellows and they chose one topic for the first-ever debate for Pediatric Cardiology fellows at NH. The topic they chose was “The role of ACE inhibitors in Post-Fontan status”. They debated well with a great deal of zeal and preparation. Congrats to Drs Shweta Nathani, Kavya Mrutyunjaya, Prashanth Patil and Dhanya Warriar – good job; keep it up!

Let me get to our new feature of the blog: The current status of controversies in pediatric cardiology.

This post, I have chosen Eisenmengarization. We shall ask few questions and check the level of evidence for each of them whenever available or get some support from literature.

Q1. The role of long term oxygen therapy:

Oxygen therapy on the first look appears to be an impressive choice. In 1985, Bowyer et al reported the ‘Effect of long term oxygen treatment at home in children with pulmonary vascular disease’ in British Heart Journal. Improved survival has been reported in a small group of children with pulmonary vascular disease receiving long-term oxygen therapy. This study was quoted to advocate the use of routine home oxygen therapy. However, a much larger study by Sandoval et al in 2001 on ‘Nocturnal oxygen therapy in patients with the Eisenmenger syndrome’ got published in the American Journal of Respiratory and Critical Care Medicine. The study showed that nocturnal oxygen therapy did not improve symptoms, exercise capacity, and outcome in adult patients with Eisenmenger syndrome. Hence, home oxygen therapy is a class IIb indication. Routine use of long-term oxygen therapy cannot be recommended. However, selected patients with associated lung pathology may benefit from oxygen inhalation. (level of evidence:B)

Q2. The role of Bosentan:

The BREATHE-5 (Bosentan Randomized Trial of Endothelial Antagonist Therapy) trial by Galie et al published in Circulation in 2006 looked at Bosentan therapy in patients with Eisenmenger syndrome. It was a multicenter, double-blind, randomized, placebo controlled study. Here, bosentan improved exercise endurance at 16 weeks, without any deleterious effects on gas exchange. As the level symptoms in the enrolling subjects increase (class III and IV), the results are likely to be earlier and better. However, in a retrospective study published by Adriaenssens T et al in European Heart Journal in 2006 titled ‘Advanced therapy may delay the need for transplantation in patients with the Eisenmenger syndrome’, no survival advantage could be demonstrated among those who received the newer therapies in comparison with those who remained on standard care. Many of these patients out of a cohort of 43 had class II symptoms. Hence, use of Bosentan is a class I indication. Bosentan may be used in patients with the Eisenmenger syndrome with class III symptoms to improve exercise capacity and hemodynamics. The long-term benefits of any drugs in this syndrome should be evaluated on the basis of long follow-up periods. Results of studies with this design are not available at present. (Level of evidence:B)

Q3: Role of Anticoagulation

Broberg and colleagues have done extensive research on this issue. In one of the articles titled ‘Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger’s syndrome: a clinical dilemma’, published in Heart in the year 2004, they showed the tendencies in such patients for the development of pulmonary arterial thrombosis. This was followed by another study of theirs published in the Journal of American College of Cardiology in the year 2007, titled ‘Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary blood velocity’. These studies pointed at the advantages of routine anticoagulation in this subset of patients. However, the risk of bleeding episodes cannot be negated at the same time. One needs a fine balance between use and monitoring. Since the risk of bleeding exceeds the benefit in the potentially unmonitorable subgroup, the routine use of anticoagulation falls under class IIb indication. The routine anticoagulation is still remains controversial in patients with the Eisenmenger syndrome. Marked blood hyperviscosity, altered chest tomography suggesting pulmonary arterial thrombosis, and chronic atrial flutter or fibrillation are findings that may point toward the need for anticoagulation. However, chronic anticoagulation should be avoided if adequate monitoring is not feasible. (Level of evidence:C)

Q4: Pregnancy and Eisenmengarization

Yentis and colleagues in 1998 did a systematic survey in this regard. Their article titled ‘Eisenmenger’s syndrome in pregnancy: maternal and fetal mortality in the 1990s’, published in British Journal of Obstetrics and Gynaecology showed a substantial risk of mortality to both fetus and the mother. The same opinion was shared by Diller and Gatzoulis in the year 2007. The article titled ‘Pulmonary vascular disease in adults with congenital heart disease’ published in Circulation reiterated the same opinion. The risks of pregnancy should be explained to women with the Eisenmenger syndrome as soon as the diagnosis is confirmed and the options for contraception should be given. Thus, avoidance of pregnancy becomes a class I indication. Early termination of pregnancy is recommended. Careful monitoring and specialized follow-up of inevitable pregnancy in selected cases is advised. (Level of evidence:C)

Q5: Routine phlebotomy

Perloff and colleagues have detailed this issue many times. In an article published in the Annals of Internal Medicine in the year 1988 titled ‘Adults with cyanotic congenital heart disease: hematologic management’, they documented worsening of iron deficiency with phlebotomies. They had an argument that the compensated secondary erythrocytosis is better tolerated and does not mandate routine blood-letting. In another article published in 1993 in Circulation titled ‘Risk of stroke in adults with cyanotic congenital heart disease’, an increased risk of stroke was also documented by them. Broberg and colleagues in 2006 published their data in the JACC titled ‘Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease’, showing a further reduction in exercise tolerance with phlebotomies. Phlebotomies should be indicated strictly to relieve hyperviscosity-related symptoms such as blurry vision, headaches, dizziness, arthralgia and worsening of dyspnea. In all instances, maintaining an iron-replete state is crucial. Avoidance of phlebotomy is a class I indication and the level of evidence is B.

This marks the end of this topic. In future, I intend to take up an issue of importance and address atleast 5 questions with appropriate studies and available evidence. Hopefully, it should be of assistance to anyone interested in these topics. Any further queries can be addressed in the comments box or to drkiranvs@gmail.com I shall try to address those questions on timely basis.

Any suggestions about this new section are welcome.

With this, let us get back to our regular feature: Interesting learning scenarios:

HIGH YET LOW

In cases of transposition of great arteries with small ASD and large VSD, the desaturation is usually disproportionate to the extent of lesion. We had a 2 year old with TGA, large non-routable VSD, small ASD and severe PS with SO2 of 60%. The PA pressure on cath was 15mmHg. The issue was to go for a BD Glenn shunt. On chest radiograph, the Qp was not bad and the echo showed a satisfactory pulmonary venous return. So, it is just not the Qp which is the cause for desaturation here. The more likely culprit might be the inadequate ASD. Despite the presence of a large VSD, it is the interatrial communication that more effective in mixing. Hence, the argument was to add an atrial septectomy to BD Glenn. The problem is, for atrail septectomy, the patient has to go through the bypass pump, whereas BD Glenn is done off-pump. So, one has to be categorical in showing the benefit for subjecting someone to pump. What is your experience in this issue? Please let the others know.

USE AND MISUSE

“Is the diagnositic cath an entity in the current practice of pediatric cardiology?” is a hotly debated subject. The pros and cons are numerous. However, some amount of discretion can definitely be entertained in this regard. The grey zones are in plenty and the decisions may fluctuate with regard to merit of the case. I have discussed a 2-year-old in one of my earlier posts. This kid had a large VSD, a moderate ASD and PAH. In such combination, we would expect RA and RV to get dilated. However, this child had LA and LV dilatation. The TV annulus corresponded to zero Z-score and RV was tripartitie and reaching apex. Since the child saturated 94% on room air, had plethoric lung fields and the shunts were completely left to right, the operability was not doubtful. However, the paradoxical size of RV was worrisome. Cath may give us RV EDP. How useful would this data be? Cath was done, but RV injection was not done due to severe PAH. The final data acquired showed operability. Can such caths be avoided? There was nothing of importance that could not be predicted. Finally, the surgery was the same as pre-cath recommendation. What is the opinion of the readership? How would other institutes handle such issue?

HALF OR ONE-AND-A-HALF?

We had a 4-year-old with a large VSD, smallish TV, smallish RV (not reaching apex) and a moderate sized ASD. The ASD shunted right to left. There was some antegrade flow through the muscle bundles of RVOT. On cath, the PA pressure was 12mmHg. The decision was between an isolated BD Glenn shunt against a one-and-a-half ventricle repair. The latter would definitely be a superior option, but would involve resection of RVOT muscle bundles. The effectiveness of RVOT clearance would determine whether the ASD should be kept open or not. Also, if the pulmonary valve gets inadvertently damaged in the process and starts leaking, the efficacy of Glenn would come down and the smallish RV may not take the additional load of PR. This may again necessitate the ASD to be kept open. The overall picture would indicate a delicate balance. How controlled can such procedures be? Is risking a delicate balance between a fine RVOT with no damage to pulmonary valve a superior option to a non-risky BD Glenn? How would the readership vote? Please let me know. I shall update you on the outcome of this child.

ACQUIRED RESTICTIONS

Can Tricuspid atresia with restrictive ASD live upto 8 years without any intervention? Looks like an unusual association. We did see an eight year old with this picture saturating 74% in room air. The RA pressure was 12mmHg and the LA was at 8mmHg in a tricuspid atresia IIB. The cath data was otherwise OK for single pump repair except for the low McGoon ratio of 1.5. The decision was to go for a BD Glenn shunt. The same question popped up again: Should septectomy be added? One argument was not to trust the numbers of cath data and to go ahead only with off-pump BD Glenn. The other argument was a possibility of an acquired restriction of interatrial communication and thereby giving rise to symptoms as well as the numbers shown in the cath data. Is the acquired restriction of interatrial restriction possible as a part of natural history of tricuspid atresia? Is such a thing reported or seen? Please let me know your experience.

ALL OR SOME OR NONE

It was a conflict of contrasts. We had an 11-year-old saturating 75% in room air but with surprisingly active (NYHA Class 1 symptoms) with balanced AV canal defect, DORV, d-malposed great vessels with supracardiac TAPVC to SVC-RA junction. The conflict in the clinical picture led to the conflicting opinions. The cath data otherwise suited Fontan repair, but the co-existing TAPVC was not in favour. The question was whether a simple BD Glenn be done or not. One argument was that at 11 years of age, isolated BD Glenn would only be adding to surgical risk without much benefit to an already asymptomatic child. The other argument was to do with his SO2. At 75% SO2, even a 25% cardiac output ensured from BD Glenn shunt into the Qp along with some antegrade flow from RVOT would be far superior over a period of time than leaving him alone. So, rerouting the TAPVC to LA and a concomitant BD Glenn was to be considered. Of the three choices (Fontan OR BD Glenn with TAPVC rerouting OR nothing) which would you favour? Do you have any different logic to justify your stand? Please let me know.

PEDIATRIC CARDIOLOGY PEARLS:

96. Systemic arterial desaturation is present to some extent in every patient with tricuspid atresia because of complete admixture of the systemic and pulmonary venous returns. Hypercyanotic spells can occur in patients with tricuspid atresia as a result of either a decrease in the size of the VSD or infundibular narrowing. (Dick M, Fyler DC, Nadas AS. Tricuspid atresia: Clinical course in 101 patients. In American Journal of Cardiology in the year 1975 page 327)

97. Scimitar syndrome is associated with other anomalies, including hypoplasia of the right lung, anomalies of the bronchial system, horseshoe lung, secondary dextrocardia, hypoplasia of the right pulmonary artery, anomalous arterial connection to the right lung from the aorta, and pulmonary sequestration. (Gao Y, Burrows PE, Benson LN, et al. Scimitar syndrome in infancy. In Journal of American College of Cardiology in the year 1993 page 873)

98. In 1942, Streeter proposed classifying human embryos in 25 age groups, or horizons, each representing 2 days of embryonic life. He later omitted the last two stages because he thought that stage XXIII could be considered the last stage of embryonic development. This approach has been generally accepted for determining the age of human embryo. (Streeter GL. Developmental horizons in human embryos: Description of age group XI, 13 to 20 somites, and age group XXII, 21 to 29 somites. In the journal Carnegie Institutes Contributions to Embryology in the year 1942 page 211)

99. During fetal life, the partial pressure of oxygen to which the ductus arteriosus is normally exposed is 18 to 28 mm Hg. An increase in pO2, as occurs with ventilation after birth, constricts the ductus arteriosus in mature fetal animals. (Heymann MA, Rudolph AM. Control of the ductus arteriosus. In the journal Physiology Review in the year 1975 page 62)

100. Two major types of vascular anomalies were defined: Hemangiomas and malformations. Hemangiomas are benign tumors of infancy that demonstrate endothelial cell hyperplasia during the postnatal period (proliferative phase) and that undergo spontaneous regression over a 5- to 8-year period (involutive phase), without metastasis. Vascular malformations result from errors of vascular morphogenesis; they grow commensurately with the child and exhibit a normal rate of endothelial cell turnover. Vascular malformations are subdivided anatomically into groups based on the predominant anomalous vascular tissue: Capillary, lymphatic, venous, arterial, or a combination of vessel types. (Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. In journal of Plastic and Reconstructive Surgery in the year 1982 page 412)
With this, we wind up this post. I would be interested in your comments on the new section. Please use the comments link. Click on ‘comments’ to open a comments box and post your writing in it. Or you can also use my email id drkiranvs@gmail.com to put up your comments- both careful and caustic are welcome!

Regards

Kiran

2010-10-16T22:49:00.000+05:30

Dr Kiran welcomes the readership to the Pediatric Cardiology blog of Narayana Hrudayalaya, Bangalore. This post will see the review of one of the bestsellers of current generation, followed by interesting learning scenarios and the pearls. First: the book review.

“Sir, can you review ‘The Secret’ in your blog?” asked one of my students.

“Well; thanks for the suggestion, but I am not reviewing fiction”, I told him.

He looked a bit sad. “Sir, I mean Rhonda Byrne” he said slowly.

I did feel sorry for myself. It was stupid on my side to respond without the complete data. I should have asked him for complete details before discounting something as fiction.

“I am really sorry for the mistake. I will definitely review the book “The Secret” by Rhonda Byrne sometime in my blog. Thank you a lot for the suggestion.”

He felt happy. I felt lost out!

It is very common to make movies based on novels. But, this is rare- A book based on a movie! Rhonda Byrne achieved it in her own signature style. Both hits; one more than the other!

Most of the TV buffs would not forget the series “World’s Greatest Commercials”. This series was from Australia and many hooked on to it for the nice insight it provided for the creative genius and out-of-the-box thinking it provided. The concept and execution of programme was credited to Rhonda Byrne, a stoic lady with lots of common sense.

Despite all her credentials, she did find herself all time low at a point. Her business had unexpected changes and was at the brink of collapse. At the same time her father- her pillar of moral courage for years- passed away. Rhonda had hit a devastating low. It was at this time her daughter gave her a copy of the best seller titled “The Science of Getting Rich” by Wallace Wattles. The book was an anti-thesis for her. It seemed to counter everything she believed. Yet, there was a charm in the work and explanation. She could not stop thinking about it. She decided- “Why not? Anyway nothing else can go wrong. Why not implement the principle of this book in my life? Let us see the result, for I have nothing else to follow either!” The result was magical- both personal and financial.

During the process of reading and implementation, Rhonda felt that she had found a secret. She wanted to spread it. But how? She decided to make use of her existing contacts in television. Her modus operandi was simple. She would go to US and interview every teacher, write, scholar, Guru or professional speaker who was likely to know about the Secret. The interviews were compiled with background narration and edited sleekly. It was never meant for cinema halls or television, but for private circulation of like-minded people who were interested in the knowledge of Secret. It would be available on internet download in its partial version and on DVD on complete version. It was a runaway hit. Word-of-mouth publicity worked the best and the sales virtually exploded. There was no looking back for Rhonda.

When it was decided to make a book out of the movie, it was not a strategy to capitalize the success of the movie. Rhonda decided that the book should have its own soul independent of the movie. Those who have not seen the movie should also experience the book. Thus, “The Secret” remains a long homage to the thinkers and authors who inspired Rhonda: these included Charles Haanel,
Robert Collier, Wallace Wattles, Genevieve Behrend, Denis Waitley, Jack Canfield, and Neale Donald Walsch to name just a few. There are lot of narrations from the own experiences of author herself.

So, what IS the SECRET?

Rhonda says it is the “law of attraction”. To elaborate: whatever one thinks about or put attention into, becomes reality in life. You attract things, people, and situations that are of a similar “vibration” to you. The universe is essentially energy, and all energy vibrates at a certain frequency. And each person is vibrating at a particular frequency, existing as an energy field within larger energy fields.

Sounds Greek; but Rhonda explains.

Going by the contemporary logic, it is useful to imagine oneself as a transmission tower. The baseline vibration of the tower is determined by the thoughts and feelings of the person. The only difference is, unlike the regular tower, the human towers can broadcast these vibrations into the universe beyond space and time. By changing the vibrations by appropriate thoughts and feelings, one can attract better people and circumstances in life.

Let us see an example: People who are wealthy think only thoughts of more wealth. “They only know wealth, and nothing else exists in their minds.” Even most people who have made a fortune then lost it become wealthy again before long. This is because they remain focused on abundance all the time, despite current circumstances. The law of attraction MUST deliver to them the equivalent of their dominant thoughts.

So, there is a caveat. How about the negative things that often rule your thought process without your control? People who discover this law would worry more about the effects that their negative thoughts. So? But Rhonda is categorical: affirmative thoughts are many times more powerful than negative ones. “Just proclaim to the Universe that all your good thoughts are powerful, and that any negative thoughts are weak.” There is thankfully a time delay or buffer between your thoughts and their coming to fruition, which allows you some space to refine what you really want. It is only a matter of practice.

Rhonda advises the use of a 3-point creative process to get the best out of the Secret.

1. Ask the universe—you must be crystal clear about what you want.
2. Believe—act, speak, and think as though you have already received what you have asked for.
3. Receive—feel great that it is coming to you. Feeling good sets up the necessary vibration to manifest the desire.
Rhonda uses another simple example: It is as if placing an order in a restaurant. You place the order and wait for it to get delivered. There, you have the faith on humans. Can’t you develop a stronger faith on the “all-delivering” universe? Similarly, when you have placed an order in the restaurant, you would never go into the kitchen and find out how it is done. That takes away the entire purpose of “hassle-free” eating. You have to follow the same ideology here. Don’t worry how the universe provides it to you. Just have faith that it WILL provide it to you.

“The more time you invest in feeling good, the Secret works better for you”. How to do that? As Charles Haanel has put it in his masterpiece “The Master Key System”: Start living in a state of love and gratitude for everything around you. This is enough to create vibrations that that attract better things in life. It is vital to elevate how you feel in any moment, since when you have negative feelings you are blocking all the good that the universe wants to give you. The Secret should teach to make gratitude a way of your life. Affirming that you are surrounded by plenty ensures that plenty more comes your way. Give thanks for everything when you get up in the morning and before turning in at night, and watch your outlook on life and circumstances change. Instead of thinking that life is a struggle, start believing that things come easily to you

Is it all mystical rubbish? We would love to get cynical and discard the idea as pile of non-provable stuff. The Secret includes a quote attributed to Buddha: “All that we are is the result of what we have thought.” Again, this is a rational, rather than a mystical, concept. The circumstances you find yourself in today, if you are not happy with them, are not “you”! The only reason anyone is not living the life of their dreams is that they are thinking more thoughts about what they DON’T want than what it is they DO want.

The majority of people who buy The Secret probably do so with an improvement of their finances in mind. However, there are also chapters on health and relationships, and how using the law of attraction can change the world. The book and the film may be seen as works of marketing genius that have made their creator millions, but plenty of people testify to their powerful effect, attracting many intelligent people who are interested in the link between mind and physical manifestation.

Get your hands on the Book or the DVD. It will surely leave you with a “feel-good” sensation atleast!

With this review, I am winding up the book review part in the blog. I had a clear reason for the book reviews. I had gone back to some of these books when I was down with dejection for various reasons in the world. It is generally what most of us go through. Each of those reasons are like forced ‘bitter pills’ which depress you more than anything else. The list need not be produced!

In the early phase of the career, the concentration is on acquiring knowledge and skills with minimal concentration towards money. In the last phases, it is acquiring fame and power. It is the middle phase which is problematic. The financial needs do not match the earnings many times. Yet, despite all the personal issues, it is important to remain sane in medical profession. Letting the mind wander may come in the way of decision making. I have seen people who could beautifully isolate the work and personal/ professional problems. Somehow, I could never do it. I had to reach the books for the rescue. The books did a wonderful job for me. They gave me the solace that I badly wanted. I thought of generalising peace obtained. That was the whole reason why the reviews of these self-help books came up. However, I felt that the needs of the blog demand more scientific discussion. Hence, I am stopping the book reviews. Still, if someone has made any attempt to write a review on any book, please send it. I shall post those last ones for all. Thanks to my friends who did review a book for the blog and for the list of books that I have received. Sorry for letting few friends down! It is probably for the greater interest!!

With this, let us get back to the regular section: Interesting learning scenarios

PROSTINS AND ANTEGRADE FLOW

Is there a role of prostins when the antegrade flow into the pulmonary arteries is reasonable in cases of cyanotic hearts? We had a 2-month-old baby with Tetralogy. The antegrade flow PA was well documented. Despite this, the infant was desaturating. There were no pulmonary issues to explain the problem. On documentation of a restrictive PDA and no good reason for the desaturation, we considered a trial of Prostins; and it worked! The saturations did improve quite well, but desaturaion would ensue as soon as the supply of prostins tapered. That prompted us to go in for a BTT shunt. The question is: Is there a way to predict the need of additional blood flow into the lungs? As seen with this baby, the “visually sufficient” amount of antegrade flow was not enough to carry on with the saturations. Is there a thumb rule or a scientific way to predict the sufficiency of Qp in cyanotic hearts? Let us know your experiences.

ROSS WITH NO HOMOGRAFT?!

This one was an interesting. The question was brought up by our senior fellow, Dr Shweta. When a Ross procedure is done, we replace the pulmonary valve by an orthotopic homograft. How about leaving the RVOT without homograft and allowing free PR as in transannular patch repair of TOF? The annulus is still intact here unlike a severed annulus of TAP. So, the tolerance is likely to be better. This also ensures that the problems associated with homograft can be avoided. Why not extend the logic of TAP to this scenario and go on? I did not have the correct answer. The logic does not sound very good superficially, but not bad either! Is there any data or experience with this scenario? If anyone has thought of anything in similar lines, please let us know.

GOOD ONE OR BAD TWO?

Corrected Transpositions indeed create interesting discussions. As a convention, we have been using single ventricle repairs for cTGA with non-routable VSD with PS. Except for the issue of routability, the ventricles would have supported 2-pumps easily. Is the conventional repair of converting such anatomies to classical cTGA (VSD closure with LV to PA homograft) underutilised? One of our senior surgeons always quote that the best of single pump repair is inferior to any 2-pump repair. Is this analogy OK with cTGA also? It is said that the recent studies with insufficient numbers performed have reported minimal outcome differences between systemic LV and systemic RV. But, when the actual discussion comes to management plan, BD Glenn et al is decided! What is the experience in other centres? Please let us know.

RHEUMATIC CHAMBERS!?

We know the rheumatic involvement of heart valves for ages. Historically, development of the science of congenital heart passed through the route of rheumatic hearts. RHD still remains a major issue with developing countries. The pancarditis pathology of RHD is well understood. However, does it affect the overall geometry of chambers in a different way? For example, give the same quantity of mitral regurgitation, does the LV of RHD different from that of non-RHD etiology? When it comes to the management, we go by the LV volumes as a marker of progress. But the mitral valve of RHD has different operation technique and outcome compared to the non-RHD group. Are we extrapolating the volume analogy of MR for RHD cases also just for the lack of sufficient data? Are there any studies to understand the LV volume differences in RHD Vs non-RHD cases with same amount of regurgitation? If anyone has done any work on it, please enlighten us.

ENLARGING THE VSD

We had earlier discussed the issue of conal tissue resection as a part of VSD routing. Now, the question goes to the other side. The option of enlarging the VSD is considered by surgeons who tackle daring things like REV. It is definitely a risky business. We recently came across a 10-year-old who had undergone enlargement of VSD for routing issues. She was fine in the immediate post-operative period and was later lost for follow up. When she returned after 3 years, we were surprised to find a severe LVOT obstruction at the junction of IVS and its patch take-off. The VSD enlargement which was sufficient during the post-op period had outgrown somehow. Is this known? Can the enlarged VSD grow on its own? Is the natural history different for the native VSD against the enlarged VSD? Is this predictable? The amount of data we have is not adequate enough to draw conclusions. If anyone has seen these scenarios earlier, please let us know.

PEDIATRIC CARDIOLOGY PEARLS

91. Between the two papillary muscles of left ventricle, the medial papillary muscle is more vulnerable to ischemia. (Voci P, Bilotta F, Caretta Q, et al. Papillary muscle perfusion pattern. A hypothesis for ischemic papillary muscle dysfunction. Circulation journal in the year 1995 page 1714)

92. In 1916, in World War I England, Sir James Mackenzie presented a paper on Soldier’s heart- a form of heart trouble to which young soldiers were particularly susceptible, manifested in spare, thin young men with great vasomotor instability, easy fatigue, breathlessness, and pain over the region of the heart. Systolic murmurs were frequent, and heart size was normal. Exertion produced undue rapidity of the heart. During the latter part of World War I, the soldier's heart terminology was changed, and Thomas Lewis introduced the term effort syndrome into the British literature. In the US of A, they renamed the condition neurocirculatory asthenia, which became the official terminology in the U.S. Army and also was incorporated into the early nomenclature lists of the New York Heart Association. (Wooley CF. Where are the diseases of yesteryear? Da Costa's syndrome, soldier's heart, the effort syndrome, neurocirculatory asthenia, and the mitral valve prolapse syndrome. Circulation journal in year 1976 page 749)

93. When the degree of valvular pulmonary stenosis is severe enough to cause a decrease in fetal right ventricular output, a larger-than-normal atrial right-to-left shunt is established in utero. This condition has been termed critical pulmonary stenosis. (Freed MD, Rosenthal AR, Bernhard WF, et al. Critical pulmonary stenosis with diminutive right ventricle in neonates. Circulation Journal in year 1973 page 875)

94. In children with Pulmonary atresia with intact Interventricular septum, the tricuspid valve is invariably abnormal- may represent a continuation of the abnormality of distal structures, ranging from extreme stenosis to regurgitation. (Choi YH, Seo JW, Choi JY, et al. Morphology of tricuspid valve in pulmonary atresia with intact ventricular septum. Pediatric Cardiology journal in year 1998, page 38)

95. Infants of diabetic women have tenfold increased risk of developing Pulmonary Atresia with VSD. The infant of a mother with insulin-dependent diabetes had nearly a 20-fold increased risk of developing PA-VSD. (Ferencz C, Loffredo CA, Correa-Villasenor A, et al., eds. Malformations of the cardiac outflow tract in genetic and environmental risk factors of major cardiovascular malformations. The Baltimore-Washington Infant Study 1981 to 1989. Armonk, NY: Futura Publishing, 1997 page 102)
This brings us to the end of another post. Please let me know your comments and criticisms. I am planning to take up some of the controversies in pediatric cardiology and review of available literature about them with the current recommendations. There is another idea of reviewing one paper or anomaly with every post with the data as current as possible. One of these would replace the existing book-review section. Any fresh idea is welcome and would be duly acknowledged. Please use the comments link. Click on ‘comments’ to open a comments box and post your writing in it. Or you can also use my email id drkiranvs@gmail.com to put up your comments- both colourful and caustic are welcome!

Regards

Kiran

2010-10-01T23:58:00.000+05:30

Dr Kiran welcomes all of you back to the present post of blog. One of my friends has taken a special interest in the “Book review” section. He is a management person, very familiar with the books described prior. Many times in the past I have heard him talking about the need for excellence and tragedy of incidental leadership in our country. He often spoke of his motto in life: “A school for teaching leadership.” Although ridiculed by many, he stuck to his views. Over years as my understanding is getting a bit mature, I feel the desperate need of my friend’s school!

I received an email from the same person asking me to introduce a book for our readership. He holds this book in a very high regard, which is interestingly titled “On Becoming a Leader”.

This post, we shall see the same book suggested by my friend. The book whose contents are described as “groundbreaking”! The author of the book is Dr. Warren Bennis PhD, founder and Distinguished Professor of the Leadership Institute, Marshall School of Business, at the University of Southern California in Los Angeles. At young age, Dr Bennis was influenced by teachings of Abraham Maslow. He found a mentor in Douglas McGregor, the common interest being Maslow. The term “Bureaucracies” was taken very seriously by him and critically analysed. Dr Bennis coined an antonym for the term. He used “Adhocracies” to imply the constructive angle. For his work on Group dynamics and new organizational forms, he obtained a PhD from MIT. Along with Harvard, Cincinnati he also served as faculty for IIM Kolkota. His most famous work till date remains “On becoming a Leader” published in 1989 and since then has been translated to 13 different languages across globe.

On Becoming a Leader is written in a personal style. It keeps asking the reader how one can make leadership a habit of existence while the world around the individual becomes a blur of change. In provides some of the extraordinary insights into the concept of leadership. The theme of the book can be summarised as “True leaders are not interested in proving themselves, they want above all to be able to express themselves fully.” The latter component becomes pertinent because only by continually seeking their fullest expression, leaders will be able to engage in periodic reinvention. Proving oneself is likely of a limited or static selfish value. Dr Bennis emphasises that life is not a competition but a flowering.

Another path-breaking concept is “Structured education and society often get in the way of leadership”. The statement may look clichéd, but Dr Bennis explains. “What we need to know gets lost in what we are told we should know.” Real learning is the process of remembering what is important to you, and becoming a leader is therefore the act of becoming more and more your true self. Leadership demands THAT unique vision of yours be accomplished. When people protest that they can’t lead, or don’t want to lead, they are usually thinking of management and giving speeches. But leadership is actually a challenge to escape mediocrity which in reality enables to lead oneself with the team. Thereby, the concept of leadership undergoes a paradigm shift.

As per Dr Bennis, the real leadership involves some of the hardcore ethics and needs. Just to list a few:

• Continuous learning and never-dying curiosity.
• A compelling vision: leaders first define their reality (what they believe is possible), then set about “managing their dream.”
• Developing the ability to communicate that vision and inspire others to follow it.
• Tolerating uncertainty and taking on risk: a degree of daring.
• Personal integrity: self-knowledge, candor, maturity, welcoming criticism.
• Being a one-off, an original: “Leaders learn from others, but are not made by others.”
• Reinvention: to create new things sometimes involves recreating yourself. You may be influenced by your genes and environment, but leaders take all their influences and create something unique.
• Taking time off to think and reflect, which brings answers and produces resolutions.
• Passion for the promises of life: a belief in the best, for yourself and others.
• Seeing success in small, everyday increments and joys, not waiting years for the Big Success to arrive.
• Using the context of your life, rather than surrendering to it.

The last point is interesting. Dr Bennis highlighted the link between self-knowledge and business success. Although it took some time to seep in, it is now a Globally recognised entity. At present, most of the successful leaders are not just satisfied with doing a job or running a company, but are trying to find an outlet for their personal vision of the world. Many companies attract best of brains by offering them good money and feeling of prestige. However, those companies which retain these best brains offer them more than that: the chance to make history. That goes very well with the motto of Amazon.com: “Work hard, play hard, change the world.”

Dr Bennis was probably one of the pioneers in the western world to break the myth of “Leaders are born”. He showed the west that Leaders can be MADE. He consistently argued against leadership thrust upon someone. These people have no guarantee of making good. If they do, their genetics or genius is lauded. If they don’t, they doom all their followers into deep well! Hence, leadership is more of a choice involving the leader himself first!

We often see the leaders on whom the responsibility is thrust upon, getting their vision narrowed. They try their best to enjoy the power and preferences to acquire what they lust the most. They cannot lead simply because they have no vision to empower everyone to achieve their best. When someone points to their fallacies, they always have the escape words ready: “I never asked for this; you gave it to me!” One of the tragedies of modern democracy is the choice people are given to choose. The paradigm shift that Dr Bennis provided in his masterpiece should work for the new age leaders. It may be ideal to end the summary with the own words of author, “What is true for leaders is, for better or worse, true for each of us. Only when we know what we’re made of and what we want to make of it can we begin our lives—and we must do it despite an unwitting conspiracy of people and events against us.”

Please let me know your comments on the above review. If you also have any book of interest in your collection, please write a similar review on the book. I shall post your review on your name. Otherwise, please inform me about the book. I shall try to review the book in the future posts with acknowledgements to the informer!

With this, let us get back to our regular feature: Interesting learning scenarios

ARTERIO-ARTERIAL

We have often seen collaterals to the ipsilateral arm in cases of classical BTT shunt. But, is it possible to have collaterals to subclavian artery from descending aorta in cases of modified BTT shunt? We came across on such situation in an 8-year-old with a blocked right BTT shunt. Angio demonstrated a collateral arising from descending thoracic aorta, scaling upto the right subclavian and opacifying its proximal course. Why should there be collateral to a vessel with good flow and same pressure? Is it because of partial block in earlier BTT shunt? Can such an event be an impetus for development of collateral? If anyone has seen this scenario earlier, please let me know.

NO INFLOW CAVITY

Is it possible to have a ventricular cavity when there is absolutely no inflow to it? In other words, can there be LV cavity if there is mitral atresia intact IVS? Similarly, can there be a RV cavity in cases of Tricuspid atresia intact IVS? One of our senior surgeons ruled out such a scenario until we showed this! We had this 5-year-old with Tri Atresia with intact IVS and confluent branch PAs fed by a non-restrictive PDA. The PDA flow not only fed the branch PAs, but also trickled back into MPA and into the RV cavity via pulmonary valve in a retrograde fashion! Is this pattern seen by anyone? We could not recall having seen such combination anytime in the past. Should we call this Tri Atresia A (no inflow) or B (Normal PA pressure on cath) or C (PDA non-restrictive). I am planning to send the echo and cath images to someone like Dr PSS Rao, who has done great deal of work on Tri Atresia. Meanwhile, please let me know if you have any data on this scenario.

ANATOMY Vs STREAMING

How can one be sure that the disproportionate desaturation often found in some of the children is secondary to mixing or streaming? We had a 5-year-old with complete AV Canal defect, TAPVC and multiple VSDs. His great arteries were transposed with severe PS, but reasonably good sized branch PAs. He saturated about 78% in room air. Since the VSDs were too large to create a single piece of IVS and 2 proper pumps, it was decided for single pump repair. Now the questions would be: How to explain the desaturation when the branch PAs are good sized. Since it was also a TAPVC, would streaming matter? Also, how to establish the suitability for single ventricle if the PA entry is not possible? In the presence of so many odds, is it better just to open midline and go with the idea of on-table measurements of pressures and decide between BDG Vs BTT shunt? When we talk of streaming as one of the causes of disproportionate desaturation, do we consider the fluid dynamics with the available anatomy or is it just an extension of some hypothesis? Are there any studies to establish the streaming? Please let m e know your ideas on these issues.

DOUBLE ARCH PDA

How to distinguish between a diverticulum of Kommarel and double aortic arch when a PDA is found with the latter? We had a 5-year-old with the combination of double arch and PDA. Both dorsal and ventral arches gave rise to 2 neck vessels each. The PDA arose from the dorsal arch. The child surprisingly had no symptoms. Now, how to establish the dominant arch? Is the establishment of dominant arch a prerequisite for surgery? What is the least complicated way out? If you had any experience of having handled such a scenario, please let us know.

PEDIATRIC CARDIOLOGY PEARLS

86. The cause of supravalvular aortic stenosis is reduced or abnormal expression of the elastin gene on chromosome 7q11.23. Large arteries such as the aorta and proximal pulmonary arteries have high elastin content in the media and therefore are more commonly affected than smaller arteries. (Stamm C, Friehs I, Ho SY, et al. Congenital supravalvar aortic stenosis: A simple lesion? In European Journal of Cardiothoracic Surgeons. In year 2001 page 195)

87. Atleast one out of 4 children with congenitally correctedTGA will have dextrocardia or mesocardia (In Anderson’s textbook of Paediatric Cardiology. Edinburgh: Churchill Livingstone, 1987 page 867)

88. The incidence of co-existing anomalies increase multiple-folds in TGA with VSD than TGA with intact ventricular septum (In Kirklin’s textbook of cardiac surgery, New York: Churchill Livingston, 1993 page 1383)

89. The highest number of neonatal deaths in the first week of life due to a cardiac cause is by Hypoplastic Left Heart Syndrome. (Samanek M, Slavik Z, Zborilova B, et al. Prevalence, treatment, and outcome of heart disease in live-born children: A prospective analysis of 91,823 live-born children. In the journal Pediatric Cardiolohy in year 1989 page 205)

90. The individual leaflet sizes of mitral valve are a matter of debate in many cardiology centres. However, it is understood that the posterior leaflet is longer at its base and shorter in its basal-to-apical length than the anterior leaflet; both have approximately the same area (Ranganathan M, Lam JHC, Wigle ED, et al. Morphology of the human mitral valve. II: The valve leaflets. Circulation journal in year 1970 page 459)

This would bring us to the end of another post. Please let me know your comments and criticisms. Any fresh idea is welcome and would be duly acknowledged. Please use the comments box or my email id drkiranvs@gmail.com

Regards

Kiran

2010-09-28T16:27:00.000+05:30

This is Dr Kiran welcoming you back to the present post of blog. The book review is being received well. Some of the issues written in the learning scenarios of last post had eyebrows of few people rising! But, as my colleague Dr Prem Alva put it, “What’s the purpose of blog if it does not even allow open discussion?” I agree with him. I have retained the post as it stood.

Let us get back to the present post. As usual, we shall see the book review, learn from few patient scenarios and the pearls.

This post, we shall see one of the massive best-sellers of contemporary literature. It is authored by Dr Richard Carlson. It was the number one bestselling book in the USA for two consecutive years. Yet, this is not a theoretical adage for perfection. It was a simple book with about 100 strategies and ideas for avoiding trouble whenever possible! Each idea is briefly elaborated in a short essay or anecdote. It has its basis from an ethereal spiritual law of Hinduism and Buddhism – of taking the path of least resistance whenever and wherever possible for betterment. These strategies have proven their worth for millions across the globe.

There is an interesting story behind the book. One of Dr Carlson’s books had a foreword written by best-selling author, Wayne Dyer. For his next book titled “You can Feel Good”, Dr Carlson requested Dyer to write another foreword. Somehow, it did not happen. But, by mistake, Dr Carlson’s overseas publisher used the previous book’s foreword of Dyer in the new book too! When the copy reached Dr Carlson, he was taken aback. He wrote a lengthy apology to Dyer and spoke of his efforts to get the copies taken off the shelves. In a few weeks time, he received a note from Dyer. It was simple two line letter which read:

“Richard. There are two rules for living in harmony. #1) Don’t sweat the small stuff and #2) It’s all small stuff. Let the quote stand. Love, Wayne.”

That started another super-seller for Dr Carlson!

Good heartedness is what one would appreciate in this book. The present day culture and wants have a crushing demand on our psyche. Lecture from a Guru or a meditation camp on a weekend do not have effects lasting for more than 2 days. What we need is the extension of that feeling for a longer time and possibly for a lifetime! How to do this? This is the question with which Dr Carlson starts. The refreshing approach that “Don’t Sweat the Small Stuff” takes is in not worrying about having bad feelings. “Don’t try to get rid of them, but do try to put them into a larger context” is one of the advices!

Some of the tips that really have amazing effects are:

• Rising early the day, long before the spouse and kids, would entitle oneself to “golden hour.” One can read, meditate, or think about the day in peace and solitude. This single act of becoming an early riser has revolutionized life for many people who practise this religiously!

• Do superachiev ners run through constant emergencies and never relax? On the contrary, Dr Carlson says, frantic thinking and constant movement leach motivation and real success from the lives. He emphasizes that peaceful and loving does not equate oneself to apathy! If inner peace becomes a habit, there is ease in the way one would achieve goals and serve others.

• Do you have the habit of interrupting others or finishing their sentences during conversation? Quit it! This simple measure puts you into lot of peace and happiness with yourself.

• John Lennon had said “Life is what happens when we are busy making other plans.” With attention to the present moment, fear—being associated mostly with an imaginary future—tends not to exist. Tomorrow’s troubles usually sort themselves out. This is a very powerful principle and repeatedly emphasized by people like William Osler and Dale Carnegie.

• If something really worries you a lot at the moment, please ask yourself “Would this matter to me in a year from now?” You may end up laughing at the problem immediately. All the energy that would have gone futile on thinking and getting angry would become more constructive and lively!

• What is the definition of accomplishment? Dr Carlson amazes you by telling that it is not an external thing. Just staying calm and composed during an adversity itself is an accomplishment. He advises to practice and recognise small but significant accomplishments everyday.

• How do you react when someone disapproves you? You may either become very angry and burst out or you may get very emotional and sob silently and get gloomy. Is there another way? Dr Carlson advices us to “respond” to the situation than “react”! He suggests acknowledging the disapproval and re-analyse. The retention of energy is a positive move and lets you take better decision.

• How do you measure your priorities? People who have read Randy Pausch (The Last Lecture) may have some ideas. Dr Carlson does a similar suggestion in his book: Imagining oneself at own funeral! Some relevant questioning at that moment might be - What sort of person was I? Did I do the things I loved and did I really love and cherish those close to me every day? And so on. That allows oneself to cut down the sweat on small things and concentrate on real issues of importance: love and happiness.

The best aspect of the book is its brevity. It is recommended for those who keep saying they don’t have time to read stuff. Feelings are the product of thoughts and by becoming more conscious of own thinking, one can get in a position to change thoughts and therefore feelings.

“Not sweating the small stuff” is simple to follow although many disagree in the first thought. A celebrated psychologist has recognized “Not sweating the small stuff” as a key feature of what he called the self-actualizing person, a person who has given up pettiness for an unusually wide view of the world and life. That is what the book emphasizes in a joyful way.

The beauty of the book is that any page can be opened and read. Though it has nearly 100 strategies all explained in a simple, brief way, practising even a couple of them might be worthwhile and may be life-changing.

Let me know your views on the book if you have read it. If you haven’t yet, I strongly recommend you to lay your hands on one and let me know how you feel after reading it. If you have any additional comments on the book, please post them in the comments section or to my email. I shall post them on your behalf.

With this, let us get back to our interesting learning scenarios.

SPLIT SECOND SOUND AND TETRALOGY

“Is it theoretically possible to get a split second sound in TOF physiology?” was one of the questions asked to me in class. I was taking a class for cardiology diploma students and this question popped up suddenly. I could not recall anytime when I had heard a split S2 in TOF. But, the question is hypothetical here. The logic tells us that any obstruction beyond the valve capable of producing a significant RVOT obstruction should be producing a split S2. Hence, a large VSD with significant bilateral branch pulmonary artery stenoses can theoretically produce the picture of TOF physiology with split S2! If any of the readers has seen such a picture, please enlighten rest of us!

AORTIC DOUBLE EDGE!

How can a dysplastic aortic valve with severe stenosis and moderate regurgitation in a 6-year-old tackled? We had this scenario recently. This dysplastic aortic valve is obviously not suited for ballooning. The difference in the annuli of aortic and pulmonary valves was too big for Ross. Repairing a dysplastic aortic valve is as certain as toss of ten coins at the same time! The fair option would be aortic valve replacement. But, the age was crucial. It is indeed a double edged sword situation. Since the patient was asymptomatic, we thought we could buy some time. How would one approach such scenarios? What is the experience of other institutes? Please let us know if you have handled such scenarios and your experiences on outcome.

OUTING McGOON

The surgeon friendly McGoon ratio may not be the favourite of everyone, but for the lack of anything simpler, it continues to be in use. The logic of measuring abdominal aorta diameter at diaphragm is well explained. However, are there any fallacies to it? We do McGoon ratio and Nakata index for all cath studies that require PA anatomy. The disparity is often striking, especially in VSD with pulmonary atresia. Does the hemodynamic logic applicable to two semilunar valves different from that of one semilunar valve? In other words, does the abdominal aorta get unduly dilated when both the ventricles drain into aorta? What is the observation in other centres? Please let us know.

APICAL VSD AND RV

“In a moderate sized apical VSD, along with LA and LV, even the RV increases in size” was the statement by one of our senior consultants. Is it correct? Just because blood traverses through the body of RV in systole, can the RV get dilated? My understanding is that ventricles get dilated only if they behave like capacitance chambers. In other words, unless the shunt blood stays in a ventricle during diastole, it cannot get dilated. Wherever may the location of VSD be, RV cannot be a capacitance chamber as blood entering into RV via VSD never stays there during diastole. Is there any literature or case study mentioning RV status in apical VSD? Any personal experiences in this regard? Please let us know.

FLOOD OR DROUGHT

Single pulmonary artery is another situation which leads to hair loss in cardiologist and surgeon (due to constant scratching of head!). We had a situation in which 11-year-old had virtual single pulmonary artery physiology. The RPA was about 8 mm and the LPA was hardly 3 mm at narrowest point, becoming 5 mm distally, looked hypoplastic overall. The child saturated 80% at rest. There were high pressure collaterals supplying lungs. One of our surgeons felt the need for a BTT shunt to RPA as possible final palliation. The supposed logic was the growth of PAs and symptomatic improvement. However, does this justify the risk/benefit ratio? There are existing high pressure pulmonary collaterals and tight stenosis of proximal LPA. Wouldn’t the BTT shunt flood right lung and accelerate PVRI there? The LPA is less likely to grow even with the BTT shunt to RPA. I felt that our surgeon had another explanation but could not express it then. I sought his time for further discussion of this scenario. In the meanwhile, what do you think of this scenario? Is the BTT shunt better or worse? Is future drought better than the present flood? Please enumerate your experiences about this condition.

PEDIATRIC CARDIOLOGY PEARLS

81. Nearly one fourth of patients with arch anomalies but without intracardiac defects have 22q11 deletion (McElhinney DB, Clark BJ III, Weinberg PM, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. Journal of American College of Cardiology in year 2001 page 2114)

82. When PAPVC and ASD coexist, the hemodynamic picture may be similar to that of uncomplicated ASD. The left-to-right shunt may be large. This shunt is the result of anomalous drainage of most of the blood from the anomalously connected lung and of anomalous drainage of half or more of the blood from the normally connected lung via the ASD (Swan HJC, Hetzel PS, Burchell HB, et al. Relative contribution of blood from each lung to the left-to-right shunt in atrial septal defect. Circulation journal in year 1956 page 200)

83. The development of the left innominate vein at the 7th week of gestation usually is followed by the involution of the left SVC (LSVC), which becomes the ligament of Marshall (Marshall J. On the development of the great anterior veins in man and Mammalia. Philosophical Transactions of Royal Society of London in year 1850 page 133)

84. In tricuspid atresia, it is common for the VSD to decrease in size, thereby changing a patient's classification. (Rao PS. Natural history of the ventricular septal defect in tricuspid atresia and its surgical implications. British Heart Journal in year 1977 page 276)

85. In tricuspid atresia, ECG in patients with diminished pulmonary blood flow often shows small R waves with shallow Q waves (Davachi F, Lucas RV Jr, Moller JH. The electrocardiogram and vectorcardiogram in tricuspid atresia. Correlation with pathologic anatomy. Am J Cardiol 1970;25:18)

That brings us to the end of one more post. “Is it necessary to involve controversy to get more people reading your stuff?” is one of the most pertinent questions asked in many quarters. I feel the quality of content has longer lasting punch than the temporary titillation of controversy. Let us just keep up the former than the latter. This blog is meant for dissemination of meaningful knowledge. The contents and questions will always be kept in that way. Any contribution to improve the existing stuff is most welcome. Please use comments box or my email id drkiranvs@gmail.com Thank you all.

Regards

Kiran

2010-09-17T23:10:00.001+05:30

Dr Kiran welcomes all the readers of this blog. Thanks for the responses I am receiving for book reviews. I have decided to continue the book-reviews till I get another flash! This week, let me introduce another “eye opener”! This would be followed by our regular features: Interesting learning scenarios and Pediatric cardiology pearls. By the way, anyone who has read and appreciated Dr Riesh Sukharamwala’s account in the last post- please reply. He would be encouraged to write more!

Let us get to the book first:

“Do you have Kiyosaki’s book?” asked my Boss one day.

“Yes, Sir. I do. Would you like to read it?”

“Please send it to me.”

When I read Robert Kiyosaki for the first time, only the novelty factor impressed me. However, over a period of time, I think I matured to the contents of book! It is something I would not hesitate recommending to anyone. It is titled “Rich Dad, Poor Dad: What the Rich Teach Their Kids about Money… That the Poor and Middle Class Do Not!”

The best part of the book is that it does not teach anything about market ventures, real-estate, dot com or anything similar. It concentrates on individual discipline and private attitudes towards money. When most of us equate money with wealth, Kiyosaki differs. He teaches us the difference between money and wealth. Money is a result of wealth or real value, and sometimes only a symbol of it. What is real is what has generated the money: a business with revenues greater than costs, a property with rent greater than mortgage, knowledge that earns royalties and so on.

The Rich Dad principle would be, “If you look for money and security, that’s all you’ll get.” You might get “money” but not find the source of money. The fundamental difference between the Rich Dad and Poor Dad is that the Rich Dad knows the difference between an asset and a liability. Anything that generates money—that actually puts it in your pocket—is an asset. Everything that takes money from your pocket without more returns is a liability! The job earnings are returns and not assets. The income coming from assets need not even have you to be around!

Kiyosaki maintains that unless one knows how to read a balance sheet, he is financial illiterate! Financial literacy, he says, is as important as word literacy. “Illiteracy, both in words and numbers, is the foundation of financial struggle.” His Sutra for getting rich is knowledge. Before making any investments, one should educate oneself on all the options and opportunities. “The more you know, the better your decisions will be!” Lack of financial education teamed with the desire for quick riches leads to disaster. “Most people, in their drive to get rich, are trying to build an Empire State Building on a 6-inch slab,” he says. The key to controlling money in his view is by controlling emotions. Becoming rich involves self-discipline and the ability to separate the emotions of fear and greed from a good investment decision.

This book makes the reader think a lot. It forces the reader to reflect not merely about their investments and assets, but about their whole attitude to work and life.

The stock market is always said to be driven by “fear and greed.” Kiyosaki claims that, for most of us, fear is the key influence in our personal economic lives. We are shaped by our attitude to money, and our attitude to money is shaped by our fear. If we could change our attitude to risk and wealth, we could begin to think, act, and live like the rich. But first we must become financially intelligent.

It is strongly advised to get hold of this book for all the simple yet strong principles it offers for enhancing your intelligence!

Please send your comments on the book and criticism on my writing on it!

Let us get back to our regular feature: Interesting learning scenarios

BANDING QUESTION

One of the most fascinating occurrences in pediatric cardiology is congenitally corrected transposition of great arteries (cTGA). Any student of medicine feels extremely fascinated when the anatomy is understood for first time. However, it takes lot many years to understand the lesion. Every child with cTGA teaches a different lesson. We had a 12-year-old with cTGA, intact IVS. The LV was obviously regressed. Cath showed LV pressure of 60mmHg against RV pressure of 120mmHg. There was a voice on PA banding and preparing LV for double switch. Surprisingly, few more people seemed to support this! I have no objective data on till what age such a venture is attempted. What is the maximum age you have come across in which such a surgery was fruitful? Is there a cut-off age till which we can attempt the 2-stage arterial switch? Please send me your opinions and experiences.

REPLACEMENT ISSUES

Continuing with cTGA, is it worth replacing the tricuspid valve in cases of cTGA with severe TR? We accept that the TV and RV are not physiologically fit enough to tolerate systemic pressures. Hence, they fail over a period of time and TR is a part of the natural history of cTGA. Is the problem with the TV or the chamber distal to it which is pumping against the systemic afterload?

THE LEFT PUZZLE

We had a scenario that could not be explained by any of us. This 9-month-old came with a large ASD L to R, moderate VSD L to R and moderate PAH. The surprise in the picture was LA and LV dilatation in the presence of normal sized tricuspid and mitral valves. The RV did reach the apex nicely from subcostal views. We are still wondering how to explain the paradox. The classical teaching is that the sizable proximal shunt always overtakes distal. In this baby with a large ASD, it should invariably RA and RV dominance. How to explain this paradox? Is this seen earlier by any of the readership? Could you offer any explanation for this? I have asked for follow up as the baby was not much symptomatic. Please let me know your experiences and explanations on this issue.

CAT(H) ON THE WALL

Cath studies of borderline values always make a nice “cat(h) on the wall”! The data can be twisted in the most convincing manner depending upon individual moods! We often see the borderline PA pressures in single ventricle physiology either making it for BD Glenn or losing the case based on the individual mood of the day! “The data is collected when patient was continuously on oxygen, hence likely fallacious”, “The numbers are when the patient was sedated and would be more when the patient is walking and talking” are some of the explanations offered for rejecting a case. Similarly, “The PA pressures may come down once we offload the ventricles via BD Glenn”, “Once oxygenated blood starts circulating, the ventricular function is likely to improve” are some of the explanations given for accepting. The problem is: one can argue on either sides of the case. Can the relative non-objectability be replaced by better objective criteria in each case? Is there a way of telling if a child is fit or not-fit for the procedure? How different is the scenario in other places? Let me know your experiences on this critical issue.

ADULT TAPVC

“No TAPVC is inoperable” is the stand of one of our senior surgeons who must have done TAPVC rerouting in thousands of children and significant number of adults. But, objectively, how to p[rove this point? It is very clear that cath study is of no help in deciding operability, as denominator is indeterminable. Is the measurement of RV EDP useful? May be, but by itself, EDP cannot determine operability. Unlike other high Qp situations, pulmonary venous return is not an assessable entity here. Hence, only time can tell whether the decision of operating was appropriate. Fortunately, we do see some of the older patients with TAPVC successfully going through the scalpel and ending up with near normal PA pressures in post op phase. What is the experience of readership? Please inform the others if your views are similar or different.

PEDIATRIC CARDIOLOGY PEARLS:

76. In ASD, the P-R interval may be prolonged, especially in older patients, because of intra-atrial and sometimes H-V conduction delay, resulting in first-degree atrioventricular block (Shiku DJ, Stijns M, Lintermans JP, et al. Influence of age on atrioventricular conduction intervals in children with and without atrial septal defect. Journal of Electrocardiology in year 1982 page 9)

77. The cleft in the anterior mitral leaflet is directed toward the midportion of the ventricular septum, along the anteroinferior rim of the septal defect. In contrast, isolated mitral clefts (not otherwise associated with AVSD) are directed toward the aortic valve annulus (di Segni E, Edwards JE. Cleft anterior leaflet of the mitral valve with intact septa: A study of 20 cases. American Journal of Cardiology in year 1983 page 919)

78. With the perimembranous defect, there can be a variable degree of anterior malalignment between the infundibular septum and the anterior ventricular septum such that the aortic valve appears to override the defect. Such lesions cannot be called tetralogy of Fallot even if there is some RVOT obstruction. (Soto B, Becker AE, Moulaert AJ, et al. Classification of ventricular septal defects. British Heart Journal in year 1980 page 332)

79. In a PDA, due to increased return into the LA, left-to-right shunting through a stretched, incompetent foramen ovale secondary to left atrial dilation is a fairly common association. This should be evaluated correctly and the incidence of coexisting ASD should not be overestimated. (Rudolph AM, Mayer FE, Nadas AS, et al. Patent ductus arteriosus. A clinical and hemodynamic study of patients in the first year of life. AAP Pediatrics journal in year 1958 page 892)

80. Isolated anomalies of minimal significance are more frequent with bicuspid aortic valves (Baroldi G, Scomazzoni G. Coronary circulation in the normal and the pathologic heart. Washington, DC: Office of the Surgeon General, in year 1967)

Good news was the contribution from Dr Ritesh Sukharamwala, a consultant Pediatric Cardiologist from Surat, Gujarat. He was an alumnus of Narayana Hrudayalaya and a member of the team. You can find his early experience as an independent pediatric cardiologist in the previous post. He has promised me atleast one contribution every month from now on. Hope others would follow soon.

That brings us to the end of another post. Please send your comments and criticisms to drkiranvs@gmail.com or put them in the comments box below. I am thankful for the positive responses I am hearing for the book review. Also, as thought previously, planning a separate section for Pediatric PGs interested in cardiology. Please let me know your suggestions on structuring the section.

Regards

Kiran

2010-09-14T16:53:00.003+05:30

At last!

After more than 2 years, I have a contributor for the blog!!

Dr Ritesh Sukharamwala, who trained in our team at Narayana Hrudayalaya from Nov 2006 to July 2009 has shared his experiences as independent Pediatric Cardiologist. Please go through and let us know your opinions:

Following is the complete transcript of his experiences in first person. I have not edited a single word; there was no need for any editing. Everything seems right from heart!

Over to Dr Ritesh:

Where am I standing at the end of 1 year? A freshly passed out pediatric cardiologist

I would life to share my 1 year experience as a pediatric cardiologist in my home town Surat.

After completing my fellowship in Pediatric Cardiology at Narayana Hrudayalaya it was very clear in my mind to settle down in my home town Surat. There were lot of comments and suggestion by senior colleagues stating difficulties of establishing new subspeciality of pediatric cardiology in small town. As a pediatrician during my residency days I have faced lot of problem in treating pediatric cardiac problem. So with a vision of providing tertiary level of pediatric cardiac care in my city I started my journey at CARE hospital Surat. CARE hospital is basically cardiac hospital providing services for adult patients. To begin with, this hospital provided me basic infrastructure in the form of echocardiography machine & OPD.

I started meeting pediatrician in my city and near by areas. To begin with there was lot of apprehension - whether this small city will accept this new subspeciality? Will I get patients? Will I be able to provide quality work? Whether I am capable? Will I be able to do interventions independently?

I started getting 1 or 2 patients a day in my OPD who were primarily referred by pediatricians. There were days where I had not seen a single patient in a day. I also started doing bedside ECHO in outside hospital ICUs.

Everyday was like a new day, learning and applying something new. This was for the first time when I have no backing of my senior. There was always a check echo by senior colleague even in my last days of fellowship. I was making my all patients rely on my reports and there was no second check. I remembered my teacher said” though you have cleared your exam, your exam starts from today”. In my initial few months of my services I got out of inferiority complex and started believing in myself. By this time I was in the second month of my journey. I had a plan to focus more on outdoor work for first six months and then to think of interventional work.

But in second month itself a patient knocked the door for PDA device closure. The time to use other infrastructure i.e. cath lab had come. The patient was admitted for the procedure. My night before procedure was sleepless. At every step I wanted to be double sure. Confirming the diagnosis more and more times and checking the hardware many times. Procedure was done successfully and that was for the first time when I felt that feeling of eternity and happiness of saving somebody’s life. I realized the meaning of job satisfaction. After that I had many diagnostic cath which went uneventfully.

Second challenge came when I had a 2 kg 2 day old preterm with critical aortic stenosis but my confidence level boosted up after its success. It was a true life saving procedure. I mean it is a life saving procedure but I realized it in true sense on that day.

Pediatric cardiology program is incomplete with out a team. I inspired my surgeon to take challenging pediatric cases. I use to go to operating room, gross anatomy was confirmed on table and surgery was done as per plan. We got away with cases like ASD, VSD, PDA & TOF. I also had to play a major role in post operative care especially when my ICU staff was not trained for pediatric critical care. It was now 6 months of my journey and the time have come when I have to think in terms of treating more complex cases which needs trained surgical expertise like arterial switch operation and single ventricle pathway surgery. One of the trained pediatric cardiac surgeons was called for arterial switch operation. Everything went smoothly & child was discharged on 11th POD and doing well on follow up. That day was new landmark in the history of my town Surat when arterial switch operation succeeded. All pediatric procedures were done for the first time in entire South Gujarat and I feel proud of enjoying honour of being the first interventional pediatric cardiologist of South Gujarat.

At the end of one year I am realizing that my journey has in fact started. Today I am seeing around 150 patients in month out of which 2-3 patient gets ready intervention and 3-4 children for surgery. I still have same feeling of apprehension when I do intervention today as every case is a new case and I don’t want to take a chance.

My majority of above said statement depicts my immaturity which I have already realized. However I firmly believe that there is no other way to success when you are alone and not backed up by your senior colleagues. I consider myself lucky to go through this phase which you will never face when you are protected by your seniors. I am trying for progress of pediatric Cardiology branch as a whole rather than individual progress. I am still learning daily and there are lots of things coming forward to learn, I mean life ends but process of learning never ends.

I am looking forward for valuable suggestion and guidance from my senior colleagues and teachers.

Sincerely,

Dr Ritesh Sukharamwala
Pediatric Cardiologist
CARE Hospital –The Heart Institute
Surat.
email: sukharamwalaritesh@yahoo.com

Please reply to the blog comments, or to Dr Ritesh or to drkiranvs@gmail.com

Regards

Kiran

2010-09-08T23:20:00.001+05:30

This is Dr Kiran welcoming all the readership to the present post. Thanks for all the response I received for the new idea of book review. Please note that the introduction to the book neither serves as a criticism nor a publicity material! I am quoting those books which no longer require any publicity. Whenever you come across these books (pavement, bookshops, library, friends’ house etc), if it kindles your memory cells and instigates you to read through, the purpose stands served. With this, let us get back to the post contents: Another book, interesting learning scenarios and pearls.

In this post, we shall see one of the all time bestseller. It is read by millions, discussed in forums and one of the books that has actually made an impact on the corporate world. It is authored by Dr Spencer Johnson. He used to work for a company called Medtronic, which manufactures cardiac pacemakers and prosthetic heart valves. His other works include “One minute manager” (coauthored with Kenneth Blanchard). Dr Johnson used to tell a motivational story to groups of people over 20 years. On insistence from Blanchard, Dr Johnson wrote the story in the form of book. It is claimed to have sold 12 million official copies (and lots of pirated ones!) The book is titled “Who moved my cheese?”

The story of the book is simple. It all happens in a maze with four characters: Two mice: Scurry and Sniff, and two “little-people,” Hem and Haw. In the beginning of the story, they have found a huge source of their favourite food, cheese and all iz well! Hem and Haw move their houses to be near the cheese and made the cheese centre of their lives. What they fail to notice is that the cheese is getting smaller. One fine day they come to see that the entire cheese is gone and get devastated.

The story bifurcates from this point into two perspectives. The mice, Scurry and Sniff, quickly accept the loss of the cheese and start exploring the maze for other sources. The little-people, however, refuse to accept the fact. They have built far too much around the source and find it difficult to move on. They start feeling that they are the victims of some kind of fraud or theft. The more they stick on and crib, worse the matters become for them. They achieve nothing better than hunger.
Meanwhile, the mice move on and find new cheese.

The message of the story or “Take home points” is nicely represented as Graffiti that the mice write on the walls of maze.

The simplicity of the book is in not taking oneself seriously. One should develop an ability to laugh at themselves in the moments of predicament. It will have a surprising effect of liberation from the situation. The book, in a way, reiterates the Buddhist philosophy of attachments making oneself weaker and the need for dissociating from one’s attachments!

Everyone’s afraid of change, more for the people who think some point is final and try to “settle down” at the place. However, change is perhaps one of the basic facts of existence. Everyone would like to assume that change would not happen to them. Denial of this basic rule prevents them from sniffing the cheese often and making way for the search of new cheese. A certain level of risk and adventure makes the life worth living. By letting the life adapt to new risks, one would involve change and the latter loses its horror. The mice in the story realize that breaking through the fears makes them free. Ironically, the little-people continuously seek security and always live with the fear that they may lose it!

The book less than 100 pages is something worth a read and contemplation. Try getting your hands on it!

Let us get back to our routine section: Interesting learning scenarios

UNDOING TRAGEDY

I have discussed this in earlier blogs also. But the question keeps coming back. If the cardiac lesion is perfectly suitable for 2-pump repair, but someone earlier has already put the child on single pump pathway by Glenn few years back, how successful can we be in undoing the problem and bringing the patient back to 2-pump pathway? Our surgical team feels that the chances of undoing a Glenn go down with time. Thus, once the child has spent a couple of years with Glenn, it may no longer be fit for 2-pumps. How is the experience in other centres? Is this scenario seen often? Please let me know your ideas and experiences on it.

DELAYED UNMASKING

Shones complex is something that has been haunting us for a long time. Someone quoted recently that Shone was either a nobody or a fictional person, for despite describing such a complex array of disorders, his name does not feature anywhere else in history! We had a 6-month-old with Shones physiology. The supramitral membrane was resected, LVOT cleared and Coarctation repair done. Immediate post-op period did not show any major issue on echo. However, after few days, the mitral valve problem slowly started unmasking itself. We found a small gradient across the mitral valve inflow, which kept increasing with time. The hemodynamics kept on deteriorating. We could virtually think of no other solution than opening again. The general condition of baby did not permit a major undertaking again. Is this phenomenon known? Why does such a thing happen? How do the inflow gradients across mitral valve change in such scenario with time when the LV function all along was reasonably good? Is there anything that we might have missed? Please let us know your experiences.

COLLATERAL EFFECT

We had a 4-year-old boy with a strange issue. This boy had TAPVC with very limited echo windows. SO2 was 60%. One of the consultants felt a possibility of superior-inferior ventricles and possible single ventricle physiology. On cath, we found multiple collaterals from proximal SVC! The RV levophase showed the pulmonary venous confluence draining into distal SVC with no obstruction throughout the course. How to explain the SVC collaterals? Were they pop-offs of the high pressures in proximal SVC, as seen with borderline Glenn shunts? Why should there be such a picture at all prima-facie? One explanation was from TAPVC draining to distal SVC. There may be a possible high pressure at distal SVC resulting in collateral formation proximally. However, cath failed to demonstrate any pressure gradient. Since the PA pressures were high, he could not go on Glenn, more so in the setting of pre-existing SVC collaterals! If you have seen SVC collaterals in a non-Glenn setting, please share your experiences and causes.

GOOD REGURGITATIONS

One cause for prepared LV in cTGA intact IVS is said to be severe TR. But, in practicality, how many times do we come across this as cause? There may be stray case reports of this event. Considering that virtually every cTGA develops TR, can it be taken as a cause? Should we not take this as an exception than a rule? Should we not evaluate for other reasons for LV preparation? We do see many MR children in OPD. How many sever MR do we recall having caused severe hypertension? Don’t we teach the students to check for other causes before committing on MR as the cause of sever PAH? Should we not think same analogy in cTGA cases also? One argument was the bowing of intact IVS towards LV changing LV geometry and preparing it. It looked far-fetched to me. Please let me know your takes on it.

PRIMUM PROBLEM

In how many situations do we see severe PAH in an infant with isolated primum defect in a non-Down setting? One of our senior surgeons remarked that he is not surprised with this combination. Is it true? The conventional teaching appears to be against the scenario we faced. In this 7-month-old baby, the baseline Qp/Qs was 0.5! Atleast, I could not recall having faced such situation earlier. Please let us know if you had any such events and how you handled this.

PEDIATRIC CARDIOLOGY PEARLS

71. In dilated cardiomyopathy, the sphericity index, derived from comparing the long-axis left ventricular dimensions in diastole and systole with the chord from the mitral annulus to the apex in diastole and systole, has been used to quantify. The value should be <0.66. If it approaches 1.0, indicating that the chamber is rounded instead of elliptical, a dilated cardiomyopathy is likely (Tani LY, Minich LL, Williams RV, et al. Ventricular remodeling in children with left ventricular dysfunction secondary to various cardiomyopathies. Am J Cardiol in year 2005 page 1157)

72. Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction (Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of Cardiomyopathies. Circulation journal in year 1996 page 841)

73. Myocarditis is a process characterized by inflammatory infiltrate of the myocardium with necrosis and/or degeneration of adjacent myocytes not typical of the ischemic damage associated with coronary artery disease. This definition does not take into account the underlying cause (Wynn J, Braunwald E. The cardiomyopathies and myocarditis. In: Braunwald E, ed. Heart disease: A textbook of cardiovascular medicine. Philadelphia: WB Saunders, in year 1997 page 1404)

74. The cardiothoracic ratio on chest radiography is predictive of mortality in patients with dilated cardiomyopathy (Manolio TA, Baughman KL, Rodeheffer R, et al. Prevalence and etiology of idiopathic dilated cardiomyopathy (summary of a National Heart, Lung, and Blood Institute workshop). Am J Cardiol in year 1992 page 1458)

75. In hypertrophic cardiomyopathy, mitral valve systolic anterior motion and outflow obstruction may also develop in childhood, associated with progression of LV hypertrophy in the presence of a developmentally small outflow tract (Panza JA, Maris TJ, Maron BJ. Development and determinants of dynamic obstruction to left ventricular outflow in young patients with hypertrophic cardiomyopathy. Circulation in year 1992 page 1398)

That brings us to the end of another post. For various reasons this post was delayed. Let me try and get them on time in future. Please send your comments, criticisms and confrontations to drkiranvs@gmail.com or put them in the comments box below. I would like to know the need to continue the book review. Also, planning a separate section for Pediatric PGs interested in cardiology. Please let me know your suggestions on structuring the section.

Regards

Kiran

2010-08-19T23:48:00.002+05:30

Dr Kiran welcomes the readership to the new post. In the last few posts, I tried some innovation in the form of anecdotes which carry greater meanings to life. Thanks for all the positive responses to them. In fact, a couple of inspired friends tried their hands on writing some stuff and with minimal editing from my side, they did a fabulous job. Great going!

From this post on, I am rendering a new idea to the readership. I will be introducing few books in next few posts. These are the books that made a great impact on me in the formative years and still rule lot of my thinking process. I had made some systematic notes on such books all these years. I will be presenting one book per post. We shall start with a small introduction to the author, go on to the works and take one classic of the author. We shall see the essence of the classic and if possible, read some excerpts from the book. If the readership has any different strategy in getting introduced to a book, the advice would be most welcoming.

Let me start with the book I admire. In the year 1998, Dr Howard Cutler, a renowned psychiatrist brought out a book titled “The Art of Happiness: A Handbook for Living”. The twist in the tale was the co-author of the book, who probably did not write a single word for the book! Many thought that the name was a gimmick and Dr Cutler just wanted a bigger publicity for his book. However, Dr Cutler maintained that the contents of book essentially belonged to both the authors. The book became a classic by the might of its contents. The role of the co-author was undeniable, for, he was the Holy Dalai Lama.

The essence of the book is simple; it says “happiness is not a luxury but the purpose of our existence”, taking away the mystical component from happiness. Another shackle breaking thing in the book is the concept that there is a definitive way leading towards happiness! It claims that achieving happiness is scientific and not by chance; it needs discipline to do that.

Some of the points emphasized in the book are:

• A basic way to happiness is to cultivate affection and connection with other human beings.

• The real source of happiness is control of your consciousness, which is impossible without a disciplined mind.

• No matter how powerful they seem, negative emotions and states of mind have no foundation in reality.

• Don’t confuse happiness with pleasure. Pleasure is of the senses and can seem like happiness, but lacks meaning. Happiness, in contrast, rests on meaning and is often felt despite negative external conditions. It is stable and persistent. While pleasures are a bonus in life, happiness is mandatory.

• Happiness is something to be developed over time.

• The fundamental nature of human beings is gentleness; people like to be altruistic if they get a chance.

• Compassion is useful. Rather than being sentimental, it is the basis of communicating well between people. Compassion is not “feeling sorry for someone” but a recognition of commonality.

• Distinguish between love based on attachment and love based on compassion.

The essence of the book is its simplicity. It gives off a sense of the lightness to life, despite all the negative things prevailing in the world. We may not understand everything about our existence; it is all the more important to be good to other beings and to leave the world a slightly better place. It should be our objective and one should not astray from it.

Let me know your comments and criticism on the book and its review. If you have any book which you would recommend to the readership, please let me know. I shall try to get that book introduced.

Let us get back to the real world now: The interesting learning scenarios!

EBSTEIN TENSION

One of the most indecisive lesions for a pediatric cardiologist is Ebsteins anomaly. Every surgeon possibly has a different approach to this lesion. But, has anyone seen an Ebsteins anomaly with severe pulmonary hypertension? Looks paradoxical, but I happened to see a report stating this combination! It was fag end of the day and this 6-day-old presented with this echo report. Unable to believe, I had to re-do the echo. To my surprise, I did find severe PAH, but the Ebstein component was missing! The STL origin was normal, but its tip was tethered to the IVS, causing non-coaptation of TV and the resultant severe TR. The lower half of the IVS was swiss cheesed. How to handle such a scenario? I have started the baby on diuretics, ACEI and asked for a surgical opinion. I shall update this issue when the baby returns for follow up.

LESSER CRIME!

What is the least morbid option in cardiac surgery? The witty answer would be not doing the surgery at all! However, this question came up a serious way in one of our meetings. This 6-year-old girl had VSD with pulmonary atresia with small confluent branch PAs and sizeable collaterals. The options would be BT shunts (possibly bilateral) or unifocalization with/without BT shunt. Considering the complexities and the practical problems in approach, the question was “which is the least morbid?” Jokes apart, it was decided to go for the latter option. I shall update the result after the procedure.

FONTAN FOUNTAIN

I can sense why surgeons are not very keen on Fontan completion. It is often said that the patient should earn his Fontan completion. We had a 4-year-old single ventricle Glenned 3 years back with all parameters favoring Fontan completion. He did quite well in the immediate post-op and was nicely extubated. However, in a matter of few days, he has developed desaturation, respiratory distress and his need for oxygen increased. The Fontan fenestration was seen shunting right to left. Added to it, there was significant pleural effusion. To make the matters worse, a saline contrast injected to a peripheral vein showed contrast in the cardiac chambers, indicating the development of pulmonary AV malformations. The last finding could not be easily explained when there was a flow from IVC to PA, indicated by the gradient across the fenestration. Should such children go for a take-down of Fontan or should they be managed conservatively? Please let me know your opinions on this issue.

BAD COMBO

We had a 3-month-old with DORV and remote muscular VSD. This combination is described in the classification, but is rarely seen. The bad part was the other lesions. This baby had normally related great arteries, severe PAH (No PS), restrictive PDA flowing R to L and coarctation of aorta. Baseline saturation in room air was 80%. Obviously, such combination is not suited for 2-pumpo repair. The coarctation would ensure rapid progress of high PVRI. Since the kid was only 3-month-old, we expected some daring surgeries and eventuality. However, our offer of Coarctation repair with PDA ligation with PA banding plus-minus BTT shunt was not taken by surgeon. The option of PA band does not exist when the baseline saturation is less. The addition of BT shunt would reduce the possibility of future single pump. Moreover, the high risk palliation is not a wise option. With all these explanations, the surgery was deferred. What is your opinion? How would you plan something for this? Let me know your takes on this.

SINGLE OR ONE-AND-HALF?

The pulmonary atresia with intact IVS is a tricky situation. Lot of variables may lead to lot of mistakes in decision making. Predicting their outcome is difficult. We had a 1 year 2 month-old boy with this problem. The TR is severe and the RV is muscle bound. RV apex is not visualized on echo. The pulmonary atresia is membranous. The contemplation of single pump was risked by the TR. One of our upcoming surgeons came up with an idea that is worth sharing. He is considering a Brock’s procedure, passing a cannula across the membranous pulmonary valve, slitting the membrane open using a balloon in the OT. This would be a semi-hybrid procedure! We are indeed excited!! I shall update the outcome in next posts. If anyone has done this before, please let us know.

DARING ATTEMPTS

After a long time, our surgeons have started doing adventures things! We did a Norwood stage 1 and the baby is doing very well. It is very encouraging and we should be doing them more from now on. On the other end, we did a REV procedure in a dTGA, VSD, PS when the VSD was deemed non-routable. Our surgeons enlarged the VSD and did a long LV to Aorta patch followed by a REV. It is very commendable and we all pray for good outcomes in these babies. How many centers are actually attempting REV? If you have any experience with it, please share with us.

PEDIATRIC CARDIOLOGY PEARLS

66. In neonatal IE most cases occur in structurally normal hearts. Although relatively uncommon, increasing numbers of cases of neonatal IE have been reported since the 1970s. This reflects the increased use of prosthetic intravascular devices and more frequent insertion of long-term indwelling central venous catheters (Ferrieri P and others: Unique features of infective endocarditis in childhood. Circulation journal in year 2002 page 2115)

67. The pericardial space, lubricated by lymph, normally contains <30 mL of fluid in the adult and considerably less in infants and children (Holt JP. The normal pericardium. Am J Cardiol in year 1970 page 455)

68. Chorea may be the sole presenting manifestation of lupus, this disease should be considered whenever a child presents with chorea (Arisaka O, Obinata K, Sasaki H, et al. Chorea as an initial manifestation of systemic lupus erythematosus: A case report of a 10 year old girl. Clin Pediatr journal in year 1984 page 298)

69. The seasonal variation of Rheumatic Fever in the temperate climates parallels that of Grp A Streptococcus pharyngitis. Both Grp A Streptococcus pharyngitis and Rheumatic Fever are more common during the winter and spring in temperate climates, but there is no consistent seasonal pattern in the tropics. Geographically, Rheumatic Fever occurs in all latitudes and altitudes (Kumar RK, Rammohan R, Narula J, et al. Epidemiology of streptococcal pharyngitis, rheumatic fever, and rheumatic heart disease. In: Narula J, Virmani R, Reddy KS, et al., eds. Rheumatic Fever. Washington, DC: American Registry of Pathology in year 1999 page 41)

70. In Kawasaki disease, generalized microvasculitis occurs throughout the body in the first 10 days of disease. Myocarditis occurs in the first 3 to 4 weeks, with mononuclear cell infiltration and edema within the myocardium and conduction system. Valvulitis may affect the mitral and aortic valves (Gidding SS, Shulman ST, Ilbawi M, et al. Mucocutaneous lymph node syndrome (Kawasaki disease): Delayed aortic and mitral insufficiency secondary to active valvulitis. J Am Coll Cardiol in year 1986 page 894)

With this, we conclude this post. Please send your feedback to drkiranvs@gmail.com Your suggestions are welcome. Please narrate your experiences with the scenarios discussed and post some novel things seen by you hitherto. Please let me know your opinion on the new section.

Regards

Kiran

2010-07-28T23:39:00.003+05:30

Dr Kiran hellos everyone. I had a good response for the previous couple of anecdote written by two friends. We shall see another anecdote now, followed by our regular features: Interesting learning scenarios in Pediatric cardiology and the popular “pediatric cardiology pearls”. The anecdote I have written this time was read few years back. I have expanded it a bit, but have retained the first-person narration of the original.

Please read and send your comments:

During my school days, there was a boy who was adored by everyone. He was not the class topper, he was not a champion sportsman, he was not an orator, he did not have great looks, his father was not the richest man in the community, he did not know any music, and he was, certainly, not a celebrity. Yet, he was liked by everyone. For the simple reason that he was good!

I wanted to know what made him so good that he was liked by all. I could sense that I could trust him; I could believe his words for he never lied. I was fond of him because he seemed to have a very simple and honest solution for every problem!

One day, he invited me to his home. I was curious to know where he lived and how his parents were. I went with him.

It was a very humble house; small, neat and clean. The utility of space was evident. I knew that my house was three times as big, but was pretty cluttered.

“Can I meet your parents?” I asked him.

“Both my parents work. They are not back till 6 PM. My elder brother has gone to tuitions and I am alone as of now”, he replied.

I recalled a joke of 10-year-old who answered the door bell of postman with a cigarette in one hand and a beer bottle in the other. “Is your dad in?” asked the postman. The kid laughed and replied, “You are a dumbo. Would you expect me in the house like this if my father was in?” asked the kid! I would have taken the opportunity of solitude to the hilt. Whereas, my friend appeared to be too good for a normal boy!

“You mean, there is no one to watch you over and still you are so good?” I asked him.

“There are certain principles that we live with in my house. They are not rules. There is no punishment if you break them or no reward if you follow them. But, I have experienced that I win applauds by following them. As for getting naughty, yes we do. But it never reaches a stage of destruction!” he replied.

“What rules…er… principles?” I started wondering now.

“Would you like to see them?” he asked doe eyed.

“See the principles?” I wondered.

“Come”, my friend led me towards a wall. There hung a sheet of parchment with neat writing in lines. Almost everyline had a different handwriting.

I read some of them:

 Do unto others as you would have others do unto you
 If you open it, close it.
 If you turn it on, turn it off.
 If you unlock it, lock it up.
 If you break it, admit it.
 If you can't fix it, call someone who can.
 If you borrow it, return it.
 If you value it, take care of it.
 If you make a mess, clean it up.
 If you move it, put it back.
 If it belongs to someone else, get permission to use it.
 If you don't know how to operate it, leave it alone.
 If it's none of your business, don't ask questions.
 If it will brighten someone's day, SAY IT!
 If what you have to say will hurt somebody, DON'T SAY IT!
 If something isn't broken, don't try and fix it.
 If you think you know it all, look around and see how little you really know.

I just kept looking at them. None of those lines seemed great. They were all simple instructions, much like what are written in the operating manuals of appliances. In fact they were nothing compared to some of the “good habits” books I had read. But the simplicity did strike.

“What are these? What exactly these lines have to do with the discipline and good behavior?” I asked him with an element of surprise.

“These are the practical principles that we follow! There is no compulsion of following them. As I told you earlier, there are no rewards or punishments attached to them. But the effect of following them is really rewarding. I do follow them very honestly. You can see what I get from everyone!”

“How do you do it?” was my question, still with the tone of surprise.

“Whenever something good strikes any one of us, we discuss the point during our dinner with all family members. The elders in the family refine the content and make it a single line principle. The person who proposed the principle will get to write the line on the sheet in his own handwriting. Once written, we all try to follow it. As you can see, there are different handwritings on the paper. Every morning, we go to the paper, read everyline with maximum concentration that we can. Even though it is a routine and I know most of them byheart, I try to make this morning affair interesting by analyzing how many of them I have followed the previous day and how many I have broken. When there is a chance for rectifying, I do it honestly. Since they are very simple one liners, we tend to remember them for long time. It has not only won me credits, it has also definitely helped in character building.”

I was struck for a while. I took me sometime to appreciate the simplicity yet the power of routine. “It is so simple to be good, but it is so difficult to be simple” was a bollywood quote I had appreciated sometime back. It seemed to manifest in the simple abode of my friend.

The above anecdote is a true life incident I had read, narrated in first person. “Trifles make perfection and perfection is never trifle” was the life motto of Michelangelo. I felt that not only every house but every workplace should also have such a chart of principles that are practical yet simple. Seeing them everyday would be inspiring and seeing the people follow it may end up whipping the “lazy bums” of the department from their inside. Would you like to have one in your home or workplace?

INTERRUPTED PROBLEMS

We saw a 9-month-old with very complex anatomy. There was a PFO shunting left to right, supramitral membrane, large subaortic VSD, complete (100%) DORV, NRGA, large conus with subaortic obstruction, small bicuspid aortic valve (Z score of minus 3), Type B interrupted aortic arch, severe PAH and large PDA continuing as descending aorta. In a cyanosed crying child, the echo took almost an hour to delineate the anatomy. The mere description of components can discourage the surgeon from any management. How to tackle such cases? It is a combination of Shones complex with complete DORV. The small aortic component does not permit too extravagant surgeries. Is there an option? Can we think of DKS in such cases? Arch reconstruction strategy would depend on what we do proximally. Is there an option of a 2-pump repair in such complex anatomies? Please tell me your opinions on this.

EISENMENGER ISSUE

There was an interesting discussion. Our surgeons maintained that the residual VSD of post-TOF patients, howsoever big the VSD might be, does not develop Eisenmengarization! Physiologically, this statement does not make much sense. But the surgeons maintain their stand, as they have not seen a single case of such physiology till now! Is there any physiological explanation for this? Is it because of the paucity of such cases that it is largely unseen? Let me know your views on it.

TO GLENN OR NOT TO

In a patient of cTGA with VSD with conventional repair (VSD closure to convert it to classical cTGA), is there a role of adding a Glenn shunt if PA pressures are acceptable? One of our senior surgeons felt that a role exists. But, logically, this would offload the MLV. The crux of the issue is MRV which is systemic. The MRV preload and afterload would not change. Why should one add a Glenn shunt in such a scenario? If anyone has any more details on this, please start a discussion.

CUT-OFF LIMIT

Is there a limit for the extent of cyanosis in admixture lesion s as against low Qp? We see huge variations in the levels of cyanosis in low Qp situations. Sometimes, even with good admixture, the SO2 would be low in few. We had a patient of cTGA with DORV and non-routable VSD. The child had SO2 of 65% but the Qp/Qs was 3.8:1! How should such instances be treated? Let me know your takes.

MITRAL PUZZLE

How far the PML mobility characterizes the MV involvement in RHD? We often see children with classical history of acute rheumatic fever with significant mitral regurgitation. Many of them have nicely mobile PML. How should we term them? Should we say that they are a combination of Acute rheumatic fever with non-rheumatic MR? How would the management change? Let me know your opinions.

PEDIATRIC CARDIOLOGY PEARLS

61. In cardiac CT scan, when manually injecting a small dose of contrast, the delay from the start of injection to start of scanning is approximated by a circulation time of approximately 12 to 15 seconds. (Siegel MJ. Multiplanar and three-dimensional multi-detector row CT of thoracic vessels and airways in the pediatric population. Radiology. year 2003 page 641)

62. In the cardiac catheterization, in the absence of a shunt, a step-up of >6% at the atrial level, 4% at the ventricular level, and 4% at the great vessel level will occur in <5% of the time. Variations of >9%, 6%, and 6%, respectively (i.e., three times the standard deviation), would be expected no more than 1% of the time, thus would be highly unlikely in the absence of intracardiac shunting. This is the mark of significant step up in cath data. (Freed MD, Miettinen OS, Nadas AS. Oximetric detection of intracardiac left-to-right shunts. Br Heart J in year 1979 page 690)

63. Shorter refractory periods may facilitate the conduction of very closely coupled impulses and could render the newborn atrium more susceptible to intra-atrial re-entry. This may partly explain the occurrence of atrial arrhythmias such as atrial flutter in the otherwise healthy fetus or newborn infant. (Pickoff AS and others. Atrial vulnerability in the immature hearts. Am J Cardiol in year 1985 page 1402)

64. Even in the current era, congenital heart disease is the most common predisposing cause of brain abscess (Goodkin HP, Harper MB, Pomeroy SL. Intracerebral abscess in children: Historical trends at Children's Hospital Boston. Pediatrics in year 2004 page 1765)

65. Kawasaki disease is a panvasculitis. Most of the morbidity and mortality in affected patients is due to coronary artery aneurysms and associated complications. (Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics in year 1978 page 100)

That brings us to the end of the present post. Please send your inputs to drkiranvs@gmail.com Since some fresh blood is getting infused to NH team, I hope to give you all something fresh!

Regards

Kiran

2010-07-27T23:19:00.002+05:30

This is Dr Kiran helloing everyone. I had a good response for the previous anecdote written by a friend. I am presenting the present one written by another friend. Thanks for the infectious zeal! This will be followed by our regular features: Interesting learning scenarios in Pediatric cardiology and the popular “pediatric cardiology pearls”. First - the anecdote: any resemblance to anyone living or dead is purely coincidental!!

Mrs Ana was enjoying her new dress in the mirror. Her husband, Mr Mike had done the payment after much reluctance. “You must dress your age and profession. You work amongst the public; these are too loud” was his argument, but she would not listen.

Mrs Ana started combing her hair, but the image in the mirror did not! She was surprised. She started waving her hand frantically, but the mirror image stood still. She got afraid and wanted to scream, but the voice did not erupt off her throat.

“Cool down”, the mirror image told her. The reflection in the mirror seemed having its own movements. Mrs Ana had no choice but to listen.

“How are your days going?” the reflection asked her.

Mrs Ana hesitated, but told “Fine”. Surprisingly, the voice had become normal.

“You are spending pretty much time with me nowadays!” said the mirror image.

“Well, you see, I am senior in the office now. My husband has come back from his posting. I have some spare time and I am trying to catch up.”

The reflection did not appear impressed. “How about your colleagues and seniors in the office? Do they have equal spare time?”

“They don’t. They still work like mad. In fact the work has increased. I pressurized the boss to hire few juniors. I clearly told him I cannot work more.”

“How is your typical day?” the mirror still did not look impressed.

“I go at around 10 am. By then, my seniors would have done the apprentices’ training session. I hang around for an hour. Then I go to the office gym. Stay there for an hour and a half. Come back and work for an hour and I go to lunch. After an hour of lunch break, I tell the others that I have to go to bank. Spend another hour hanging around at various places. I come back to the workstation by 3.45PM. By 4, I tell them that I have to attend to my family and leave for the day.”

“Very interesting” said the image sordidly. “How about your seniors?”

“As I told you, they work like mad. They start at around 8.30 AM, train the apprentices, start the work, go on till 2 PM. After the lunch, they continue till 5 or 5.30 PM. Few go on till 7 PM also.”

“How do you escape so easily then?”

“You know my previous boss. She was from my own community. My husband was working outside and my kid was young. So, out of sympathy of being a single mother, she gave lot of relaxations to me. She also paid me extra money as my husband was not earning. Both of us together made one of my colleagues a jackass and made him do all my extra work. We kept telling him that I am taking a pay-cut and he gets relatively more money than me. That idiot believed us and did all my work!! Stupid fellow.”

The image started getting blurred and came back. “You know, legally single mother is the term used for divorced or husband-dead ladies with children, unless they are unmarried and have adopted a child.”

“I do, but my stupid colleague did not” giggled Mrs Ana

“Do you justify what you did was correct? What would you have done if someone else had duped you like this?”

Mrs Ana was stern. “First, what I did was entirely wrong. Second, I would have kicked any person who would have done the same to me. After all this, I think I can still get away because I think I have killed my conscience. But the surprise is, why am I telling all the truth to you that I had hidden inside me all these while?”

The mirror image smiled. “Your first assumption is wrong. I AM your conscience. Second one is right. You can cheat the entire world but you cannot lie to your own conscience. The decision to make me reappear guilt-free or to make me vanish forever is left to you.”

The mirror image faded away leaving an awestruck Mrs Ana.

The above anecdote refers to all the black sheep who wish to outsmart their peers with the help of superiors who misplace the priorities. Work culture and ethics are relatively new terms in the western work-front, but are age-old in the Indian systems. There are numerous anecdotes in our epics and history on how an individual should work holding on to ethics and not cheating the final witness: the one’s own conscience. It is secondary whether such people get punished or get their due for wrong deeds in the outer world. It is probably more important to keep our own conscience free of guilt. The problem is in dealing with such people. They become a blemish and bane on the system. Such liabilities bring down the overall morale and work efficiency. When such characters go unpunished, they either simply spread the infection of indiscipline to others or make the honest workers rebel against the system. Once a critical number of such people are reached, the system collapses. It is for the people at power to decide whether such black sheep should be allowed to exist or perish in the system. Let us know how you deal with the “black-sheep” of your firm.

With this, let us get back to our regular feature: Interesting learning scenarios

A BANDED QUESTION

It is maintained that the best time for PA band in any infant with PAH for future single ventricle pathway is by 1 month. Although many surgeons would like to wait for some more time, the general opinion is for 1 month of age. Are there any studies which support this? What is the natural history of infants who were banded for future SV physiology between 1 month and 6 months of age? We often come across such children. One observation is the disparity in the mean PA pressure and ventricular EDP in such children. We generally get a bit liberal with mean PA pressures and plan a BD Glenn for them with the idea that the pressures may come down with the Glenn shunt. Few advocate tightening of PA further. What is the general consensus on this? Please send in your institution policies.

TAKAYASU DI(E)LEMMAS

It was a learning experience. We saw a 12-year-old girl about 3 months back. She had MVP with mild MR and trivial AR. The LV was disproportionately dilated. We did a detailed clinical examination and did not find any structural anomaly. The anemia was evident. Blood investigations showed anemia with dimorphic blood picture. We advised hematenics and follow up after 3 months to see the response. When she came for follow up, we could clearly see the clinical evidence of Takayasu arteritis. The disease process probably had shown some evidence of its presence, but we could not pick up at the inception. By the time we could pick up, the disease had shown its effects. Is there any way we could have diagnosed the problem at the beginning? Please let me know if you have found any methods.

SPINE Vs CEREBRUM

Continuing with the same issue, how effective are the interventions done in patients with Takayasu arteritis with activity in end stage? Although my seniors are optimistic, I am yet to come across any single kid with end stage Takayasu arteritis intervened and made it to another year. It is true that the morbidity comes down for few months, but the mortality factor does not seem to change much. Considering that isolated balloon dilatation is of limited value and cost of stents is usually not bearable by the poor family, it is largely an ethical question. One junior colleague of mine probably had a better expression: “We spend only spinal cord for managing any medical problem of a patient because we would have burned up our cerebral cortex for thinking the financial and social implications of the problem for the family!” Should there be a consensus for the limited finance set-ups for managing the problems whose outcomes are questionable? No one probably has enough time to think of how the family suffered to arrange the finances to treat a fatal condition with negligible outcome. The family would not have understood either the problem or the treatment, forget the chances of success. In a very high-volume centre, where is the time for one-to-one interaction? Such might be the problems of the third-world, but these are the issues which maintain us in the third-world!

BANDING THE HOMOGRAFT!

This one was a real test. We had a 7-year-old who underwent VSD closure with RV to PA homograft about 2 years back outside. Few small additional muscular VSDs were documented and were probably hoped to get spontaneously closed over time. The child was lost for follow up and presented to us one day with failure. The muscular VSDs appeared sizable. On cath, there was a significant step-up. However, the lesion was typically swiss cheesed and there was no single sizable lesion. Together, the hemodynamic effect was significant. How to handle such scenarios? The VSDs were so small that they could not be closed individually. Since it was a homograft, the option of PA band also did not exist. How to deal with such a scenario? Please let me know your options on this.

TERMINOLOGY

How would you classify tricuspid atresia in the setting of cTGA as per Kuhne’s?! The left sided TV would be atretic and the great arteries would be L malposed. Should we call it type 1 or type 3? Although many classifications are accepted for tricuspid atresia, the most used classification does not involve this scenario. Please let me know your take on it.

PEDIATRIC CARDIOLOGY PEARLS

56. In neonatal IE most cases occur in structurally normal hearts. Although relatively uncommon, increasing numbers of cases of neonatal IE have been reported since the 1970s. This reflects the increased use of prosthetic intravascular devices and more frequent insertion of long-term indwelling central venous catheters. (Ferrieri P, Gewitz MH et al: Unique features of infective endocarditis in childhood. Circulation 2002 page 2115)

57. Genetics in CHD have curious angle of research. Investigators from the Baltimore-Washington Infant Study analyzed the rate of precurrence – which is the number of currently affected relatives at the time of birth. These studies demonstrate substantial rates of familial disease and suggest that CHDs may not be as sporadic as once thought. (Boughman JA, Berg KA, Astemborski JA, et al. Familial risks of congenital heart defect assessed in a population-based epidemiologic study. Am J Med Genet 1987 page 839)

58. Although currently an unproven hypothesis, the potential neurodevelopmental advantage to be derived by the affected neonate who avoids acidemia may, in the long run, prove to be the most important long-term salutary effect of prenatal cardiac diagnosis. (Lavrijsen SW, et al. Severe umbilical cord acidemia and neurological outcome in preterm and full-term neonates. Biol Neonate 2005 page 27)

59. Two kinds of atrial receptors have been described. Type A receptors fire during atrial contraction and respond to changes in atrial pressure, and type B receptors fire during ventricular systole and respond to changes in atrial volume. Type A receptors stimulate and type B receptors inhibit sympathetic activity. These stretch receptors provide feedback to the hypothalamus and inhibit secretion of antidiuretic hormone called vasopressin (Little RC, Little WC. The output of the heart and its control. In: Physiology of the Heart and Circulation. Chicago: Year Book Medical, 1989 page 165)

60. The first reported case of autopsy-proven myocardial contusion was in 1764; it described a boy struck in the chest by a plate! (Akenside M. Account of blow upon heart and its effects. Philos Trans R Soc Lond Biol 1764 page 353)

With that, we come to the end of another post. Please let me know your opinions. Send in your comments and contributions to drkiranvs@gmail.com

Regards

Kiran

2010-07-17T00:17:00.000+05:30

This is Dr Kiran welcoming everyone to the new post. My last post did get me few responses: One of the doctors who read it commented on some of the practical patient management techniques he has been following for past few years. One of which was the use of frozen betadine! He freezes betadine solution in the freezer into small cubes. He uses those cubes to sterilize and prepare the skin before IM injection. The temperature numbs the skin and betadine provides the antisepsis. He says that none of his patients have ever complained of injection pain and no incidence of any injection abscess in the last 25 years! In fact, he claims that he is famous as “Painless injection doctor” in the locality.

“Not everything in medicine needs to be FDA approved” was what Dr Passi had written in his article. It is indeed true. We can innovate; we can change things for better and simple. The above example of using antisepsis is inspiring. I don’t know how many doctors are using the same technique, but I heard this for the first time. Please send me the details of any innovations that you are doing. Let us share them with the readership.

With this, let us start our anecdote. This was sent by a friend and I have retained his own style of narration. Please read this and discuss amongst your colleagues:

Mrs Fragrance was exposed to bright light, but her eyes were comfortable. She did not know where she was, for all that she could see was just bright light.

“Welcome Mrs Fragrance” said a deep, baritone and pleasant voice.

Mrs Fragrance could just hear the voice. The light was so bright that nothing else was seen.

“Where am I? Who are you?” Mrs Fragrance asked in a scared voice.

“You are at the court. This place decides your next journey. It can be the heaven or the hell” said the baritone.

“You mean, I am dead?”

“No conclusions at this place. You are at junction of unknown and uncertainty. Please answer some questions. It will be over soon.”

“OK”, Mrs Fragrance was uncertain.

“You can tell something about yourself.”

“I was working as a school teacher; I taught 4th standard students. I was very pious and God-fearing. I have lived a good life.”

“What subjects were you teaching?” asked the Baritone.

“I was supposed to teach them all subjects – like mathematics, science, history and so on. But everyday, I taught them ‘Jack and Jill’, ‘Twinkle-twinkle’ and ‘Johnny, Johnny’”

“Only those rhymes instead of all the subjects you mentioned to 4th standard students? Don’t they need better quality than that? It never occurred to you that 4th standards do not need ‘Jack and Jill’ anymore?”

“I did my best. I worked very hard to teach them the rhymes because I could not do any better. Coming to all the other harder subjects, I used to make the trainee teachers working in other departments do those difficult jobs.”

“Was it the duty of trainee teachers to do all these?” asked the surprised baritone.

“No; it was not. In fact, it was very hard for them to do my work. Still, I made them do it. I was very close to the head-mistress. I used that influence to get those trainees do my work. Since the trainees were afraid of head-mistress, they used to do it without much resistance.”

“Why did you not hire a junior teacher to do your work?”

“I could have. But, I wanted to show the administration that I was doing all the work. More so, hiring a subordinate would be sharing my income. I wanted all the profit for myself. Since, the head-mistress was close to me, I could do all this. By the time the head-mistress retired, the trainees had no idea that they were actually doing my job and did everything silently thinking that it is their work.”

“You never felt you were performing sub-optimal?” asked the still surprised baritone.

“I always did my best. I spent hours together in teaching ‘Jack and Jill’, ‘Twinkle-twinkle’ and ‘Johnny, Johnny’ to all the 4th standard students over years. You know how sharp these kids are. They refused to learn those old rhymes and few even demanded learning other subjects. I scolded them nicely and forced them to learn what I knew the best. But, some of my colleagues had objection. They felt that my teaching is inferior and referred few kids to other teachers. But, I brought this to the notice of head-mistress and penalized those stupid colleagues.”

“Mrs. Fragrance,” said the baritone. “Let me get it correct. You were supposed to teach normal 4th standard students and all that you taught them was ‘Jack and Jill’, ‘Twinkle-twinkle’ and ‘Johnny, Johnny’. You made poor trainee teachers who did not even belong to your department do all the hard work that was supposedly yours. You used the influence of your head-mistress in the entire wrong doing. Throughout your life, you have never kept the standards. Still, you convinced the management that you are doing all the work. You made all the money without even hiring an able subordinate. Some unlucky trainees were always there to do your work at the cost of their learning. You still think that you were hard working and deserved all the good things that happened to you all this time. Am I right?”

“Yes” said Mrs Fragrance. “Even though many in my own department used to joke, I was very honest in teaching ‘Jack and Jill’, ‘Twinkle-twinkle’ and ‘Johnny, Johnny’ to all the higher class students. I think I have done my job the best way. More so, I always attended churches. I used to apply leave or just scoot out of the job during working hours to attend the prayers and as many places of worship as possible. I must have attended more churches than any other person in my city! I was always pious and God-fearing.”

“Who would see your regular school work on all those days when you attended the places of worship?”

“The trainees. I used my influence with the head-mistress to make the trainees do my regular work. In fact, in my absence, they used to do all my work and the management was not even aware of my absence. I was paid even for my absence. Good terms with head-mistress are very important.”

“Final question, Mrs Fragrance” the baritone said. “Do you think you deserve heaven or hell?”

“Definitely heaven. I have sincerely taught nursery rhymes to 4th standard students with best of my ability. I have attended all possible places of worship, even at the cost of my own work and by applying as many legal and illegal leaves as possible. My head-mistress always felt that I was very good. I should not go anywhere other than heaven.”

The baritone started laughing. The laugh grew so loud that it filled the place more than the light. Mrs Fragrance started feeling a bit uncomfortable and …….

……she opened her eyes to the reality.

The above anecdote is written by one of my close friends who got inspired by the previous anecdotes I have been posting in this blog. He is a dedicated pediatrician working in a government hospital. He was expressing his concern about the poor quality of people working with him and how they were misutilising the system by the aid of their contacts with higher up officials. I encouraged him to write a story and he did. It is his first attempt and he seems to have done a good job! The point is, these underperformers exist in every system. Most of such people make themselves a liability for others. They are tolerated either due to fear of their contacts, for respect of harmony in workplace, by exploiting junior people to compensate for their laziness. Such black sheep shamelessly celebrate their mediocrity to pain the rest. One of the highly revered vice-chancellors of Bangalore University, Dr HN told once: “There are 2 classes of people; those who work and those who take the credit for the work! The battle in the second category is fierce. One would do very well in the first category.” Is there any example of how such people were set right? If anyone knows the tactics, please enlighten our readership!

With that, let us get back to the regular feature: Interesting learning scenarios:

A TETHERED QUESTION

We saw a 5-year-old with tethered STL of tricuspid valve. The origin appeared to be from the crux, but the STL effectively started off from 18mm from crux. Till that point, it was finely tethered, which could be made out on a high resolution with zooming. Surprisingly, the ATL was sail like. In a case of isolated tethered TV, does ATL also get involved? Is the ATL involvement a corroborative evidence of Ebstein’s anomaly? Please let me know your ideas about it.

VOLUMES, VOLUMES

It is always maintained that the saturations are not the criterion for BD Glenn shunt. The criterion should be the volume reduction. Hence, even when the baby with single ventricle physiology is saturating more than 90%, it may still be prudent to go ahead with BD Glenn even at the cost of some desaturation. If this is the case, why is SO2 taken as a criterion for Fontan completion? In India atleast, there is lot of resistance for Fontan completion. It is always maintained that the patient should earn Fontan completion than it is being given. SO2 plays a major role in pushing the surgeon for Fontan completion. Should we not have same criteria for BD Glenn and Fontan completion? Are the complexity and the presumed Risk/Benefit ratio the hitch for Fontan completion? Are we still at the learning curve for Fontan? Let me have your opinions on this issue.

COLLATERALS IN EISENMENGARIZATION

We had an interesting scenario for discussion. A middle aged male came with TGA, VSD, PAH. He was Eisenmengarized by opinion and natural history. However, his presenting complaint was hemoptysis. He had bouts of blood being coughed out. Cath study showed multiple collaterals. The pertinent question was: Is collateral formation possible in Eisenmengarised patient? The opinions were divided. Few were categorical that the collateral and Eisenmenger do not go together. Few had an opinion that not all the lobules of lung are equally Eisenmengered and those which are affected less might have developed the collaterals. Few felt that the pathology was different from Eisenmenger and should be evaluated, as proving Eisenmenger in this anatomy was not possible. Cardiac CT led to more complexity, as it gave a DD of sequestration too! However, sticking to the basic question, please send me your ideas on the combination of Eisenmenger and collateral formation. What is your opinion? Do you have a separate analogy or do you agree with one of the explanations offered by our team? Please let me know.

TWO POSSIBILITIES

What do you think is the suitable cut off age for a two-stage arterial switch surgery in those infants who come a bit late? We had a 2-month-old with dTGA, PFO and regressed LV. The option of 2 stage arterial switch is always there. Our surgical team is divided in opinion. One senior surgeon believes that the 2 stage arterial switch is a futile exercise and has not shown benefit in long run. He feels that the Senning palliation would be much superior to the hurried 2 stage ASO. The other senior surgeon has opposite opinion and prefers 2 stage ASO to Senning anytime. What is the opinion of the readership? Please let me know how you have been handling such scenarios.

CORONARIES FOR RESCUE

It is a rare thing for the pediatric cardiologist to chase the coronaries fervently. Barring the cases of tetralogy, TGA, Pre-Ross assessment, ALCAPA, Kawasaki and few more, the coronaries are not the area of interest. It so happened that coronaries came to the rescue. We saw a baby with VSD with two great vessels, one of which was hypoplastic. The orientation of posterior great vessel was towards left. The anterior vessel was hypoplastic with a gradient of 70mmHg. The arch vessels could not be visualized. The PA confluence was absent. We could not see the LPA. Supply to the right lung appeared to be from few AP collaterals. The arch appeared to be right sided with a right PDA. Overall, the picture of anatomy distal to semilunar valves was totally unclear. The differentiation of PA from aorta was largely dependent on the coronaries. We ended up chasing the coronaries to determine the great vessels, keeping the possibility of ALCAPA aside! If you were made to chase the coronaries as fervently as we did, please let me know the reason.

PEDIATRIC CARDIOLOGY PEARLS

51. The echocardiographic findings in boys with Duchenne muscular dystrophy correlate with the autopsy findings of posterior epicardial thinning leading to ultimate dilated cardiomyopathy. Studies have showed thinner left ventricular posterior walls, especially behind the posterior mitral valve leaflet, diastolic dysfunction, contraction abnormalities that progressed inferiorly, and temporally progressive wall thinning. (Goldberg SJ, Feldman L, Reinecke C, et al. Echocardiographic determination of contraction and relaxation measurements of the left ventricular wall in normal subjects and patients with muscular dystrophy. Circulation 1980 page 1061)

52. High-dose IVIG has been shown in many clinical trials to reduce the incidence of coronary aneurysms to <5% when administered for 7 to 10 days of the disease onset. (Newburger JW, Takahashi M, Beiser AS, et al. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med 1991 page 1633)

53. Administration of antibiotics during an episode of RF does not alter the course or severity of cardiac involvement. (Tompkins DG, Boxerbaum B, Liebman J. Long-term prognosis of rheumatic fever patients receiving regular intramuscular benzathine penicillin. Circulation 1972 page 543)

54. The most common cardiac manifestation of Juvenile Rheumatoid Arthritis is pericarditis. (Gedalia A, Giannini EH, Brewer EJ, et al. Prevalence of pericardial effusion by echocardiography in juvenile rheumatoid arthritis. J Rheumatol 1993 page 206)

55. Pericardial effusion has been reported in as many as 25% of patients infected with the HIV virus; however, large effusions are rare. (Starc TJ, Lipschultz SE, Kaplan S, et al. Cardiac complications in children with human immunodeficiency virus infection. Pediatrics 1999 page 14)

With this, we conclude this post. Please send your feedback to drkiranvs@gmail.com Your suggestions are welcome. Please narrate your experiences with the scenarios discussed and post some novel things seen by you hitherto.

Regards

Kiran

2010-07-09T22:56:00.003+05:30

Dr Kiran welcoming everyone to the present post

This time, I am not writing any story or scenario.

I happened to read something about the creativity and medical education recently and got pained a lot.

Just sharing my feelings with the readership. Please comment. Use the comments box or my email drkiranvs@gmail.com

Please go through and discuss:

MEDICAL FEST A.L.A. IIT FESTS

“Give them any challenge and they are ready to do it” was the compliment given by my erstwhile Boss. She was talking of the IT professionals she has hired for her new IT venture. The glow and pride in her eyes was very evident. “They are too good. There is something in them that is not in….” the continuity was lost as if something stuck and topic was deliberately shifted to something else. I did not miss the point. I could complete the half done sentence. We are trying to dodge the unpleasant and that is not the way life goes.

Yes, it is true that the creative energies of everyone in the world is appreciated other than doctors. In the recent issue of Indian Pediatrics, Dr Gauri Rao Passi had rightly pointed one thing. He was talking of the IIT fest and all the new ideas that got a material form and displayed with pride in the fest. It stood as the testimony of the creative and lateral thinking of the bright student community of future engineers. In entire lifetime, can anyone recall a similar medical fest? All the non-academic happenings of medical college are limited to a cultural event with Anthaksharis ruling the roost.

Where exactly the things went wrong? Till 12th std, all the students of science stream go through the same process of learning. By and large, best of these brains enter the medical field. (It was atleast true till the IT boom) After that? We see the engineering students creating their own satellite launched by ISRO, we see the law students representing the country in international law fests, we see the architecture students winning applauds in international forums for their creative designs and so on. What about medical students? The maximum I can recall any medical student achieving is a prize in an essay writing competition. What happened to those “Best of brains”?

Have you ever been encouraged to do some lateral thinking during your basic MBBS training? Were you ever confident of uttering a novel idea that might have come to your mind? Did you anytime feel some lacuna in the medical curriculum and wanted to desperately modify something? How many times could you do all these in a state of fearlessness?

There are lots of reasons for a decline in the creative thinking in medical student community. Lot of it is realized only on a retrospect. The teachers who teach the basic medical sciences are usually discarded ones. Most of them chose to stick to basic sciences because they did not get a clinical subject for their post-graduation. Their frustration is usually evident. I have seen PGs of basic sciences who tried yearly PG entrance exam throughout their 3 year PG course in the hope of getting a “better subject”. They never concentrated enough on their own subject during the most important formative years of their post-graduation. How would you expect such people to become good and inspiring teachers for the younger lot?

In the paramedical field, the good brains usually have a “good” attachment! A really good pathologist or microbiologist would concentrate more on developing their lab practice than teaching. But, the relative numbers of “better” people are certainly higher than in basic sciences.

By the time a student of MBBS reached the final year, the phobia of exams would surround to such an extent that it is blasphemous to think of anything other than the text book. Although really inspiring teachers may exist, the vision of a final year medical student fails to acknowledge it. For most of the students, a mediocre teacher who can consistently rant out the “Exam questions” or “Viva questions” is a bigger hero than the person who can teach with a systematic and scientific methodology.

In the entire period of MBBS, almost every student finds it difficult to accommodate to the consistently changing subjects, curriculum, teaching, preparation for didactic sessions, written and photocopied “notes” and so on. There is practically no one to encourage the creative thinking. By the time students reach a teacher with a creative bent, their mould would not let them move out of the self/system created boundaries.

Examination in the Indian medical education is no less than a horror. It is time for the students to recall how good or bad they were! It is time for the examiners to take revenge or reward the loyalty. Money, influence, good behavior, non-controversial nature etc find a larger place than the actual talent of the candidate. Passing in the final exam is such a big relief even for a consistent hard worker who knew their stuff well. By this time, their lesser intelligent friend in any non-medical professional field would have got his degree and a handsome 6-digit salary and a vehicle.

MBBS marks the end of a beginning. The actual ordeal starts from now. People with enough money can buy any subject of their choice in a private medical college. What was earlier in lakhs has reached crores now. People with influence can get direct seats from the biggies. Next best would be getting question papers of entrance exams for a price. The nexus is strong and powerful and in India, it takes a CBI enquiry to break the code! The other way is to move through your caste. If anyone can prove that they belong to the caste that government considers as backward, irrespective of any other factor, position or assets, the PG seat belongs to them for free. I have personally seen PG students who obtained their seat by reservation coming to the hospital in a Mercedes. Added to this, the nexus of fake caste certificates is probably impossible for even CBI to crack!!

However, the lesser mortals with no money or influence or reservations will have to go through the hard way. They have to take a “break” of couple of years in prime of their life after MBBS to “prepare” for the PG entrance exams. In this period, one has to sustain on the income of their parents or should do very low paying jobs of a “duty doctor”, which is actually a glorified clerkship in a private hospital. They fill exam forms of almost all entrance exams of the country, travel to the exam centre by a third class sleeper coach (which the Indian railways call second class), stay in cheapest of places to save money, eat roadside as they cannot afford high prices of restaurants and write exams for a handful of PG seats. The competition would be something like 700 doctors for each PG seat! With every successive year’s failure, the potential competition increases, as a new batch of MBBS pass outs would have arrived.

With all these difficulties, few get successful in acquiring a PG seat. Most of the doctors would not achieve the subject they always liked and loved. They end up in such a state that they would take the course which they detested from their core! Still, the life should go on. How long can you feed yourself with the parental earnings? How long would you keep answering the relatives who were jealous of your academic success few years back, but are very happy with you now that you are struggling and their otherwise low-performer son is earning 5-digits a month in a call centre or an IT company? It is time to prove. Success comes with hard work in any field. The PG subject you are studying does not matter as long as you are hard working!!

Right? Yeah! Life is smooth once you have got a PG seat? Yeah! Lot of things change in just 3 months. Bright MBBS doctors are made to feel that they are actually duffers. The first years get to do a peon’s job. They run around to collect the lab investigation reports, chest radiography films, arrange the glass vials for blood samples, store the blood and urine culture bottles in their cupboards for emergency use, make a note of all new instructions given by the bosses, request the proud class III and IV workers for shifting the patients to investigation rooms and so on. “Look at the cobweb in that corner of the ward. You people don’t work!” is the remark that I have personally got from my consultant during rounds in my first year of post graduation course.

Thesis is a source of pain for most of the PG students. Not only the process of conceiving and delivering it is tedious, bearing the idiosyncrasies of the “guide” is hellish. For the people who believe in reincarnation, all the good deeds done in the previous birth manifests as a good PG guide and vice-versa! I had the opportunity of seeing the brutalities in first hand. I know some of my colleagues, who were used by the “guide” to get vegetables for the house, drop his kids to the school, make the PG drop guide’s niece in scooter to her work place and get her back in the evening every day, wash his car and get it serviced, book his air tickets at the cost of PG and so on. It is not necessary to say that all the academic work of the guide is actually done by the PG, whether it is preparing slides for a class or writing a chapter for the text book or publishing a study. Should I mention that the PG gets no credit or authorship or acknowledgement for the work? Worse are the cases that I have heard, in which the female PGs have undergone more inhumane treatment and exploitation. With all this, the moment of getting the thesis signed by the “guide” goes through many more phases of torture and is a relieving moment at the end of it.

Where is the creative energy that was bestowed to everyone? Why our medical education system does systematically suppresses the creative thinking? Why is a person who attempts to talk out of the box is treated as a joker? Why do we behave as if we should not think outside the textbooks? The archival system of teaching medicine, inept or inert teachers, lack of opportunity for expressions, fear of getting jeered by the peer group and so many other issues end up in suppressing the ideas and creativity of every medical student. Our conventional upbringing does not allow most of us to rebel against the set norms.

I discussed this issue with some of the peers. “You cannot crib that someone’s wife is more beautiful”, “We are dealing with human lives; we cannot afford to experiment”, “Mere talk is waste; who is going to find a solution?” “All that you feel is right. But the remedy does not lie in the hands of a single person”, “You are burning from inside; you need vipasyana meditation to cool yourself” were some of the answers I got.

Why do we think of an improbable end point and stop a beginning? Who is asking anyone to radically change the treatments? There are so many innovations that can occur in teaching, learning, patient examination techniques, diagnostics, assessment pattern and so on. Why not involve student community in these? Just to suggest, why can’t we dedicate one hour every week for listening to the students? Why not a session for them to talk in an open forum to present their ideas on innovation? If someone is shy of presenting himself on the podium, why not keep a box in which he can drop his idea in writing, which can go through a set of understanding and sympathetic senior doctors? In every institute, there are few extra-ordinary and respected, out-of-the-box thinking doctors. Why not they form a group and encourage the student community in catering a creative angle to thinking? Once we dare to change a bit, we can think of changing this ignoble way of conducting our curriculum and assessment patterns.

There might be lot many ways of inculcating the innovation and creativity of the budding doctors during their formative years. “I was treated badly by my guide and now it is my turn to be bad with my students” is the hallmark of a wretched brain. How can such people make good doctors when they are not even good human beings? Isn’t there a need for changing this system somewhere? Till we think of solving these issues, we cannot think of medical fests with new ideas coming from “best of the brains” in the community.

Regards

Kiran

2010-07-05T21:02:00.000+05:30

This is Dr Kiran welcoming everyone to the new post of the blog. As we have seen, the primary objective of this blog is dissemination of genuine interest in Pediatric cardiology to all those who have got smitten by this charming bug! I had the opportunity of writing a lot of historical details about the subject and drugs used in this field. For the past few weeks, I have been writing a few short anecdotes which may touch the readers somewhere and may bring back some memories. None of these stories are my own. Most of them are heard, read or sent by the friends. It may seem personal if there is lot of relevance, but that is purely coincidental! With this disclaimer, I am moving on to the following anecdote which was sent by a friend. He did not specify if it is his own. But, the quality of story was worth sharing. Please go through:

Ram was 14 years old. His adolescence was more visible on his recent “don’t care” attitude. He was fond of science classes in the school and was quite good at it. He would often tease his father with the question, “Dad, what is relativity?” His accountant father was probably unaware of who Einstein was! Father used to just smile and keep quiet.

A new circus company had campaigned in their town. Ram wanted to see the circus. It was almost the month-end and his dad did not have spare cash for the new expense. Somehow, after taking a hand-lone from a colleague in the office, the father-son duo went for the show.

They were standing in the queue. A parallel lane was buying tickets from another counter. There was one group in the parallel lane. It was a big group with eight children, all under the age of 12. Ram felt that they didn't have much money. Their clothes were frayed but clean, and the children were well-behaved all of them standing in line, two-by-two holding hands in back of the man who had brought the kids. They were jabbering about the clowns, elephants and other acts they would see that evening. Ram, who had gone to a circus a couple of years back, could sense those kids had never been to the circus before. That evening looked like a highlight of those young lives.

Ram and his dad were behind a couple of people in the line when the children’s group reached the counter. “One full and eight half tickets, please” the man said. The lady in the counter told the amount. The man opened few notes of currency from his pocket and counted. His face fell sad. He counted the money again. “How much did you say the amount was?” he asked the counter lady again. He was turning pale with the answer.

Ram and his father were witnessing the scene. Suddenly, Ram’s dad pulled up a note of Rs 100, dropped it on ground and told the man in the opposite lane: “Sir, you have dropped this note. It fell from your pocket.”

The man looked at Ram’s father. His eyes were filling with tears. He meekly accepted the note and bought the tickets. He came towards Ram, held the hands of his father and told in a choked voice, “I don’t know how to thank you. These are the children from the neighboring orphanage. What the donors pay is just enough for food and clothes. They were dreaming day and night about the circus. As their caretaker, I could not resist bringing them here. I got some of my money and asked for some from my friends. Still, I fell short of some. Bless you, Sir; can’t repay your kindness.”

When Ram reached the counter, they did not have enough money for the tickets. They just walked away from the counter. After few yards, Ram’s dad held hands of his son. “Can we just postpone our programme by a week?” he asked Ram.

“No problems, dad”, Ram said. “Three things, actually. First, I can wait for a week. Second, I can do without too; I have seen the circus earlier. Lastly, today you have taught me what relativity is!” Ram said with a glee in his face.

Ram had found a new meaning for life that day. Moreover, he decided never to tease his father again.

The above anecdote is quite touching, especially for people who empathise. The community of Doctors probably has highest chances for empathy. One of the doctor friends, who does community service told me about her experience with patients wherein she had to make decision for them imagining herself in their shoes! It is a tough experience. The relativity of the issue probably depends on how many tough situation we have passed through in our life. There is the story of a man who was cursing God for not having footwear till he saw a man without legs! The cursing suddenly got transformed to praise. The quantum paradigm shift in this instance is evident. Life is relative and the meek ones need to know this well before they decide on something drastic. Understanding who is right is probably not as important as what is right. This is the biggest aspect of effective leadership, management, parenting, teaching and coaching. It's amazing how much we can accomplish if we simply focus on leaving everyone we meet in better shape than we found them.
With this, let us get back to our regular feature: Interesting learning scenarios

RIGHT PARACHUTE

We can all recall a number of parachute mitral valves. When the papillary muscle of left ventricle is alone, the entire chordae end up getting inserted to the same site and the physical picture is of a parachute with the pointing end at pap muscle and the balloon at the annulus. This is possible for the mitral valve where the site of insertion is well defined. How about the tricuspid valve which sends its chordate over a wider area? We saw a 9-month-old with all the tricuspid chordae getting attached to a single pap muscle. On searching the literature, we found the existence of such an entity. Anderson et al had reported this in a setting of TGA and Aziz et al for TOF. In our case, it was a large VSD and a small ASD. If anyone has any data on this entity, please let us know.

ATRIAL OUTLETS

Another interesting variant of tricuspid valve was seen by us. We have seen a double orifice mitral valve. However, a double orifice tricuspid valve is a rare variant. We had such a scenario in a 6-month-old wherein the TV had two openings on either side of interventricular septum. This was associated with a large inlet VSD and a small RV. The entire MV and one opening of TV were to the MLV and the other opening of TV was to the small MRV. It was an eventual single pump repair. The literature shows the existence of this condition without any other heart lesions. If you can recall any instance of noticing such an anatomical variant, please put up your experience.

PARTIALLY TRANSITIONAL

What would you term a Transitional AV canal defect in which the VSD has closed by a septal pouch? What is the terminology to be used? It is taught that the inlet VSD does not close. However, we have often seen a small inlet VSD of transitional AV canal defect closing spontaneously over a period of time and becoming a partial AV canal defect. Should we continue to call it transitional or change to partial? Tell me your views.

RIMS AND LOCATION

Of late, we have been largely successful in eliminating the need of Transesophageal echocardiography for ASD device closures. Our transthoracic echos have been found sufficient for ASD devices. In this process, we often find that the IVC rims being unsteady. Many times, the defect is not visible at all when the IVC is opened in the subcostal saggital view or in the short axis view. In such cases, we have found a mixed success rate. Is there is fool-proof method in the TTE for this problem? How many centres have actually dropped the TEE for ASD device closures? What are the experiences in those places? Please let us know.

SIDE TO SIDE!

Continuing the problems with devices, we have seen few muscular VSDs which look perfectly OK for device closures during echo evaluation. However, on the table, the LV side of the defect would be much larger than the RV side. Not only crossing becomes a problem, the size of the device to be used is also an issue. Since the muscular VSD devices are symmetrical, the LV side of the device may be too small for the defect and the RV side may be correspondingly big. Our experience with the VSD devices is not as extensive as our PDA and ASD devices. How are the other centres managing this issue? Please let us know if there is a way.

PEDIATRIC CARDIOLOGY PEARLS:

46. Sildenafil seems to be beneficial in the management of Primary pulmonary hypertension. Acute vasoreactivity studies in PAH patients suggest that sildenafil may have greater acute hemodynamic effects than inhaled nitric oxide and may further reduce pulmonary vascular resistance. (Michelakis E, Tymchak W, Lien D, et al. Oral sildenafil compared with inhaled nitric oxide in PAH. Circulation. year 2002 page 2398)

47. AHA guidelines on IE prophylaxis extend to HCM. Bacterial endocarditis appears to be virtually confined to patients with the obstructive form of HCM, with a prevalence of <1%. Vegetations most commonly involve the anterior mitral leaflet or septal endocardium at the site of mitral valve - septal contact and less commonly the aortic valve. ( Spirito P, Rapezzi C, Bellone P, et al. Infective endocarditis in hypertrophic cardiomyopathy. A good review is done by authors. Circulation. year 1999 Page 2132)

48. Levosimendan is a calcium-sensitizing agent that has been evaluated in adults with acute decompensated heart failure and in patients with chronic heart failure. One pharmacokinetic study has been performed in children with congenital heart disease. There is no increase in myocardial oxygen consumption or arrhythmias. (Turanlahti M, Boldt T, Palkama T, et al. levosimendan in pediatric patients evaluated for cardiac surgery. Pediatr Crit Care Med. year 2004 page 457)

49. In cases of myocarditis with congestive heart failure, digitalis may be used and has effected dramatic improvement in many instances. However, during periods of acute inflammation, the myocardium may be hypersensitive to digitalis, so rapid administration to achieve therapeutic levels should be avoided. (Parrillo JE. Myocarditis: Good review article on treatment. J Heart Lung Transplant. year 1998 page 941)

50. In Pediatric restrictive cardiomyopathy, beta-blocker therapy was suggested to blunt rapid heart rates in their patient population in whom significant ST-segment depression was noted at higher heart rates. However, tolerating the therapy is the chief caveat. (Rivenes SM, Kearney DL, Smith EO, et al. Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy. Circulation. year 2000 page 876)

With that, we come to the end of another post. Please send your views, opinions, criticisms either by the comments box or by email to drkiranvs@gmail.com I shall post them on your behalf. I am preparing the list of books about which I can talk in next few posts. Till then, I shall continue with the anecdotes. If you have any interesting short stories to share, please mail them to me. Your contribution would be acknowledged.

Regards

Kiran

2010-07-02T23:40:00.001+05:30

Hello everyone, this is Dr Kiran welcoming you to the new post. The objective of the blog is to develop an interest in Pediatric cardiology for curious. We learn few interesting facts and discuss few scenarios on practical issues. As previous, let us start with an anecdote which carries a greater meaning in life. The following anecdote is what everyone goes through in life. Please quote your views on it.

Mr Ray was a worried man. His peace was disturbed due to some problems at his office. He was not willing to discuss it with anyone, for he was afraid people may trifle his problems and start talking of their own. He was of the opinion that no one else could understand the significance, quality or gravity of his problem.

His wife, Mrs Ray could not tolerate the downhill condition of her husband. They had a common cousin who was a motivational speaker and well known “agony-uncle”. She sought his assistance and the man agreed to casually visit the Rays for a non-formal session.

Mr Ray did not know of these arrangements. He welcomed the cousin and both of them started talking. Mrs Ray took this opportunity to re-inform that the cousin was a well known advisor and specialized in solving problems in the work environment.

Mr Ray got interested to know the qualification. He hinted that he had a problem in his office. The cousin acknowledged the hint and told, “Would you like to discuss the problem with me? I can try and help it if you don’t mind.”

Mr Ray took the opportunity. His frustration had been piling up for a while. He thought of shooting it off.

“I don’t know if my problem sounds silly. But, it is killing from inside. I am a very principled man who sticks to discipline. If somebody breaks it, I get very frustrated. More so if I have no control on the person doing it. It multiplies if I get victimized in the process of someone else’s indiscipline”, Mr Ray ranted out.

“You cannot be abstract if you want a solution. Get me the facts straight”, his cousin told with a smiling but straight face.

“I have a colleague”, Mr Ray started. “I don’t want to name him. He was an apprentice when I joined, but he got promoted to the same position as mine in few months of my joining. He did not have the qualification for the post, but the Boss took special interest. Probably because they are from same community.”

The cousin nodded. He knew the rule: Do not break the chain of thought!

“It does not bother me. After all, they manipulated the rules of the firm. In a private firm, the rules are flexible if the Bosses agree. What bothers me is the attitude of the person I do not want to name.”

His cousin interrupted. “Let us call him ‘Nameless’. That will solve the problem of expression.”

“OK”, Mr Ray continued. “Nameless is an opportunistic and lazy person. He quoted his family problems and was scooting off from work. Later, he started quoting the health problems. After sometime, he took off for acquiring new training and qualification. Even now when he has got promoted to a higher position, he continues to do the same. His problems seem to be perpetual and he poses as if no one else in the world has any problems. He wants everyone to share the burden of his problems and there is absolutely no help from his side when the other people have problems”, Mr Ray was increasing his tone of anger and was getting breathless while saying all these.

His cousin was simply nodding. He also took some notes in a piece of paper as Mr Ray spoke.

“The biggest problem is on me. I have to bear all the unfinished work of Nameless. He simply gets up and goes home at his will, well ahead of the closing time of office. I have to stay back extra time to complete his incomplete business. When there is some extra work few days, he simply orders the peon to get all the files and leaves by the time files arrive. All those files get dumped on my table. Of course, the other colleagues also chip in, but I have to take the maximum brunt. I have complained to higher authorities many times, but somehow, they are very sympathetic towards him. They kept telling me that since Nameless does not work full-time, they are cutting a portion of his pay. My earlier Boss who was of the same community as Nameless was so moved by all the pathos uttered by Nameless, that he always gave an out-of-the-turn bonus for him! I recently found out that there was never a pay-cut either for him and my Boss used to present a white lie in front of me to keep me silent. Since then, my anger is burning me from inside.”

His cousin’s face did not show any emotion; neither he gestured any noises of sympathy. He kept jotting up something occasionally.

Mr Ray took a gulp of water. His catharsis was evident. His tone was returning back to normal slowly.

“The problem continues even today. Nameless hardly does any work and gets paid the same amount of salary as I do. Nobody cares on how his work gets transferred to others for completion. For the sake of the office pride, we silently bear the burden and finish the work. My burden is heavier nowadays and I cannot even say a word. I often feel like doing the same thing what Nameless does. I am seriously thinking of going scot-free. I will also do half jobs and put the rest on someone else. Anyway, when my boss is tolerating him, he will have no other option other than tolerating me too.”

Mr Ray took another gulp of water and kept silent.

“Any other people in your office? Any other person with similar nature as Nameless?” his cousin asked Mr Ray.

“There are lots working in my office, but as of now, only Nameless is the exception. Others are hard working. Few, in fact, keep very high standards in work. Few people are really inspirational.”

“Can I tell you a small story?” his cousin asked.

“Sure”, Mr Ray said.

“What I quote are from the Vedas - the divine Hindu scriptures. There is mention of a bird called Vyoma, whose sole aim is to reach the sun. It keeps ascending. It lays its egg en-route its journey. The egg keeps dropping all the way, but since the mother bird was at a very high altitude, the egg has to travel a long distance to reach the earth. Still on its journey down in the sky, the egg hatches and the baby bird is delivered. The baby bird starts of its ascent immediately and starts moving towards the sun. The mother bird continues its journey to sun till it burns off in the way due to the heat. As the Sun is described as a ball of fire and the bird also catches fire and burns out, they become essentially one. Do you find any message in this story?”

Mr Ray was silent. Obviously, it was above his head.

His cousin continued: “The story, as any other story from Vedas, is symbolic of life. It should not be taken in literal sense. The sole pursuit of life is to reach the excellence. It should be so powerful that your progeny picks it up as an inborn, innate nature. No external influence should affect the journey. You should rise so high that what other people talk of you should not even fall into your ears. For, people who stop their journey to talk are simply wasting their life. People with a high objective in life have only one way.”

Mr Ray was not convinced. “Is this advice practical?”

“Of course, yes” his cousin continued. “It depends on where you keep your vision. You can either be the anecdotal Vyoma bird to keep you vision high or you can be a vulture, which keeps flying high but always has its vision fixed on the dead bodies lying on the earth. Here, you are always watching Nameless and getting the vision of a vulture which probably befits the Nameless; not you. Why not keep the vision of some other person in office whom you admire? Why not simulate him? Why not try and get the standards that the other person has achieved? Why not excel and set a standard for others? Why not do the inevitable extra work as a learning experience and gain more out of it? Why should you ever think of going the way Nameless has gone? Would you tolerate yourself if you become what you detest?”

It was hard-hitting for Mr Ray. He was expecting sympathy. What he got was more than what he sought.

“So, how do you think I should rectify the mistakes in the office? Is it not my duty to set it right? Is it not correct to demand my rights for equality and peace of mind?” he asked meekly.

“As a matter of fact, it is not your duty” his cousin said curtly. “You have done your best by bringing it to the notice of your superiors. What they do is not your business. Yes, we all go through phases of injustice in life. It is true that Nameless does not deserve what he is getting. But it happens to almost everyone and almost everywhere. It is neither unique nor rare. If you keep thinking and cribbing about it, you are getting stalled in your journey towards excellence. You are wasting your time by underachieving your potential. You are a creative man. You can be of greater pertinence to the world. Only that you have to think in the correct direction.”

Mr Ray was speechless. He took sometime to digest what he heard. Slowly, he got up from his chair, shook hands with his cousin and said, “I may not agree with all that you said, but seems to make sense in a way. I shall try to implement and let you know the results”.

This anecdote is symbolic of what happens in everyone’s life. In every working place, a certain percentage of people are found who live a parasitic life on others. We used to call it 80:20 principle in the government run hospital I worked earlier. Overall, 20% of honest staff did 80% of all the work, but those 80% staff was struggling hard to get all the credit for the good work not done by them. The scenario may not be much different in any private set up either. There are always people who make a living by sycophancy; they just need a gullible boss! There are people who think being smart is better than being honest. There are people who allow their conscience to collect their full salary without working for it. There are people who are first to attend the meetings with higher authorities and take lead in presenting data for which they have not even contributed. There are people who believe in getting their work done by others by any hook or crook. Such people may present as witty, jovial, smart, wise-cracking and popular with everyone except for their colleagues who actually end up doing the pending work of such characters. But all those apparently smart qualities are largely to hide their lazy self. It is a frustrating experience to be colleagues of such kind. The above anecdote shares one way of dealing with such people. Such breed is so common that each one of us can recall such a character in our working place. How are your experiences in dealing with such people? How do you make yourself compatible in such situations? Please share your experiences with other readers. It is really good to have some solace from every possible corner!

With this, let us get back to our regular feature: Interesting learning scenarios.

SMALL INTO LARGE: POSSIBLE?

We had one year old girl with Tricuspid atresia IIB with disproportionate branch PAs. The LPA was far smaller than the RPA. This baby also had bilateral SVCs with LSVC far bigger than the RSVC. Cath data showed suitability for Glenn shunt. Now the problem was attaching the big LSVC to small LPA! We have seen BTT shunts that cannot exceed the size of the native ipsilateral PA. Is the rule applicable to Glenn too? Is it possible to attach a 10mm SVC to a 5mm PA? How are the dynamics different? The surgical team had a split opinion on this. Please let me know your take on it.

IMPROVEMENT IS PROGRESSIVE AND VICE-VERSA!

This one was learning experience. We had a two-year-old single ventricle- DILV. Cath data for Glenn suitability showed a mean PA pressure of 18mmHg. It was above the comfort level of our surgical team. We were about to negate the possibility of surgery when one of our senior surgeons (who is otherwise very conservative) opted to do the Glenn in this baby. It was a matter of surprise and we wanted to know the basis for his opinion. He explained in very simple terms that Glenn will take off about 30% of his systemic venous input into the heart and about 15% of total cardiac output in this baby. So, post-op, the PA pressures are likely to come down by about 15%, bringing it into comfort zone! It was an eye-opener for mathematically challenged people like some of us! But the logic appeared very appealing. The offloading concept of ventricles is the basis for Glenn shunt. When the criteria are made, is this mathematical calculation already incorporated? That is, do we fix a mean PA standard after making correction for the post-op reduction or not? I could not find an answer. If anyone knows this, please let me know.

LOCATION LOGISTICS

I had brought up this question earlier. In Ebstein’s anomaly if there is a communication between LV and atrialised RV, can we call it a VSD?! One more facet came up this time. It was a 6-year-old girl with Ebstein’s anomaly with the communication between LV and atrialised RV low down and had an aneurysm of STL projecting up and partially covering the defect! Can we call this “restriction of VSD”? Here, neither the term VSD nor the direction of STL limiting the flow is semantically confirmed! Can STL send the aneurysm towards the direction of RA and be still called by the same name? Let me know your ideas on it.

HOW EARLY IS EARLY?

It is tough to picture the cause/effect relationship in retrospect. We had a 2-day-old newborn with Tetralogy – absent pulmonic valve complex. It came with respiratory distress. On echo, the branch PAs were dilated. The chest radiograph showed pneumothorax on the right. The condition deteriorated in a couple of days and the neonate was intubated and electively ventilated. The repeat chest radiograph showed a pneumonic patch with no evidence of pneumothorax this time. The question is: Can absent pulmonic valve complex produce respiratory symptoms so early? Is it beneficial to get a cardiopulmonary CT scan? What is your experience of early presentations in such entities? Please let me know your take on this.

CAUSE – EFFECT

We had a 7-month-old with heart pushed to right. Echo revealed TOF with small RPA. The return from right sided pulmonary veins was minimal. Chest radiograph showed a totally collapsed right lung. CT chest done outside was reported as hypoplastic left lung. Is the lung status acquired or congenital? Is the small RPA the cause of right lung hypoplasia or vice-versa? Is there any way of determining the cause-effect relationship? Please let me know your opinion on this issue.

PEDIATRIC CARDIOLOGY PEARLS:

41. Reporting echocardiography in a systematic manner conveys meaning for management. As per the norm, right-sided and left-sided structures at each level are evaluated according to their morphology, their relative positions, their connections to proximal and distal segments, and the presence and location of shunts, obstructions, and valvular regurgitation. (Edwards WD. Congenital heart disease. In: Schoen FJ, ed. Interventional and Surgical Cardiovascular Pathology: Clinical Correlations and Basic Principles. Philadelphia: WB Saunders, 1989:281-367)

42. The only prospective control trial of Carvedilol use in children with heart failure was published by Shaddy et al in 2007. The trial showed an improvement in children less than 24 months on echo parameters. However, on composite end points of study, there was no significant benefit. (JAMA, 2007, page 1171)

43. Shepard et al in 1991 reported that 1 in 40 patients with tuberous sclerosis may die as a direct result of cardiac rhabdomyomas. (Mayo Clin Proc 1991;66:792-796)

44. When cardiac myxomas obstruct the semilunar valves, patients experience symptoms while bending forward or lying down, with relief of symptoms when standing. (Robertson R. Primary cardiac tumours: Surgical treatment. Am J Surg 1957;94:183-193)

45. Rheumatic mitral stenosis severe enough to result in symptomatic heart failure may occur in the first two decades of life in developing countries. (Agarwal BL. Juvenile mitral stenosis in developing countries: Problems and challenge. J Assoc Physicians India 1986;34:141-144)
With that, we come to the conclusion of one more post. I am really interested to know if there are any readers of this blog other than me! Please send your inputs via the comments section or to my email id drkiranvs@gmail.com The purpose of this blog is to make it interactive. Hope someday it happens!

Regards

Kiran

2010-06-26T22:48:00.000+05:30

Hello everyone, Dr Kiran’s welcome to all for the new post. The objective of the blog is to develop a dissemination of Pediatric cardiology for everyone interested. We learn few interesting facts and discuss few scenarios on practical issues.

Before we start our anecdote, let me congratulate our Fellowship students: Dr Sripadh Upadhya and Dr Vishal Changela, who finally had their exams last week! They have performed very well and are sure to pass out with flying colours. Dr Changela is a very honest and sincere worker. He has amazing patience while he teaches someone and often called “teacher material”! Dr Upadhya is likely to join the NH team. Good luck for the future of both these fellows.

Also, let me take this opportunity to welcome Dr Dhanya as our new fellowship student under RGUHS. Good luck for her stay and career.

With that, let us start with an anecdote as we have been doing in past few posts. The present anecdote was sent to me by mail by a friend. It is told in many management classes and motivation seminars. Enjoy:

An offbeat lecturer who was known for his eccentric ideas came to the class. He was supposed to take the class for the final year degree students. He carried a couple of bags with him.

He wished the class and opened his bags. The students were interested on what he would do without starting the regular class.

He got a glass jar out and kept it on the table. He started pouring glass marbles into it till the brim.

“Is this glass jar full?” he asked the class. “Yes, sir” came the reply.

“Is it?” the lecturer said. He carefully placed few more marbles on the pile till it started falling.

“Now, is it full?” he questioned again.

“Yes, sir” was the reply, more emphatic this time.

“Let us see” the lecturer said and brought out some fine pieces of stone and started putting into the glass jar. The stone pieces percolated down and settled in the spaces between the marbles.

“Now, what is your opinion?” he asked.

“The jar now seems to be full, sir” came the reply.

“Let us see”, the lecturer said. He took his bag and took out some fine sand. He poured the sand carefully over the jar. The sand occupied the spaces between the stone. The lecturer looked at the class.

The class went silent. Nobody wanted to commit.

“I know what you are thinking. The jar now is full. Is there anything I can fit in?” the lecturer pulled out a bottle of water and started pouring it into the jar, which seamlessly got accommodated!

“I have just demonstrated how the perception changes in life”, the lecturer began. “Our perceptions of ‘full’ and ‘fill’ are very relative. First time, adding one more marble would have dropped it out. But we had forgotten the spaces in between. So, ‘full’ was by the perception of marbles, not for space in the jar.”

The class was listening.

“Our life may seem quite filling with the job we do” lecturer continued, “but there is always scope for few more things. It need not be as major as our primary profession. It can be an activity, a hobby, a service for someone or anything. If the jar in this example was your life, the marbles would be your profession. As you saw, the jar was full with marbles, but was still empty. Life would not be a fulfilling experience unless you fill it with something more than your profession.”

The silence of the class was broken by a loud applause.

The above anecdote is self explanatory. I have seen people who keep complaining about lack of time for pursuing anything other than their job. However, they would nicely discuss a recent cricket match, a new movie, some odd soap series in the television and so on. Ask them about their reading habit, the answer would be a sharp ‘where is the time?’ Not that they do not find time, but they do not recognize it. Probably, reading does not feature in their list of priorities. I know how hard my present boss works. He is in the hospital by the time we reach and keeps going on even when we leave. I find it surprising how he manages time to read a lot. He seems to have read quite lot of contemporary literature. When I was in a book festival, I called him to ask if he wanted any book. He could easily tell me 3-4 books which he liked to read. Some of them were part of a series of which he had the initial volumes and had read them already. It is really commendable and not many actually know of this quality of his. It may be prudent to take a cue from this anecdote and try something new, which would be a fulfilling experience in life.

With this, let us get back to our regular feature, interesting learning scenarios:

OPERABILITY REGURGITATES!!

In a given scenario of operability decisions in echo for cases with severe PAH, the chamber dilatation and pulmonary venous return play the vital roles. For example, in a case of large VSD with bidirectional shunt on echo, we look at pulmonary venous return and LA/LV enlargement to ascertain the operability. We happened to see a child recently which put us into confusion. The child had multiple muscular VSDs which shunted bidirectional. There was severe TR with flow reversal in IVC. Hence, there was RA/RV dilatation. As the TR was severe, the forward flow across the pulmonary valve was probably compromised. This may result in low pulmonary venous return. That compromises both components of operability in echo. Is there any study on how a severe TR compromise Qp in cases VSD? Is the hypothesis of low pulmonary venous return in severe TR correct? Please let me know your take on it.

ONE MORE; ONE LESS

How frequent are the combination lesions with TAPVC? As a feature, we have seen TAPVC being a part of heterotaxy isomerisms. But when the TAPVC is the primary lesion, presence of defects other than a mandatory ASD are not very frequent. I have initially mentioned of a TAPVC with intact IAS, but a large VSD. We found TAPVC with severe coarctation of aorta recently. It was difficult to diagnose to begin with, as the flow across aortic valve itself is less in TAPVC. The likelihood of underestimating coarctation is very high. However, this child had a very clear 2-D picture and gradients diagnostic of coarctation. The combination of high Qp with obstruction to Qs is not very common. The same was confirmed in the operating room. Please let us know your experiences of such atypical combinations.

DUAL DIFFICULTIES

One more variant of TAPVC was seen on the same day as the previous scenario. A 6-month-old came with features of increased Qp. We found a dilated coronary sinus and the common confluence of pulmonary veins entering the CS. We prepared the report of cardiac TAPVC. But, the routine suprasternal view picked up something more. We could see another channel with the colour Doppler showing the flow towards the probe. We started tracing the origin of flow and it went into the common confluence. We reconfirmed the presence of dilated CS and the entry of common confluence into it. It was an instance with the common confluence of pulmonary veins communicating with more than one exit channel. In our case, it was communicating with CS and via ascending vertical vein into the SVC and RA. The combination is very well described, but not frequently seen. It reconfirmed the sayings that our seniors repeatedly told us: Always see all the possible pathways of exit in a TAPVC. If our complacence permitted us to stop at the level of CS, we would have lost an important finding of surgical implication. Let us know your experiences of TAPVCs with dual drainages.

WHY SHOULD NOT WE?!

Several logical explanations are put up on why a small PDA be closed. The risk of endocarditis and the ease of device closure top the list. In the same logic, why should we spare a small muscular VSD? The VSDs described as “maladie de Roger” are the small mid muscular VSDs with high gradient, often with a thrill but no volume overload of left heart. Such lesions have risk of endocarditis, but are too much for open heart surgical risk. Extending the logic of small PDAs, the risk can be eliminated with the device closure of such VSDs. With the learning curve for muscular VSD device closures getting sustained in many centers, why should our guidelines be changed for the favor of device closure of such lesions? Give your opinion on this issue.

THE QUEST FOR CORONARIES

One of the popular viva questions in pediatric cardiology involves coronaries. “Where all in pediatric cardiology do you need to chase the coronaries?” Usual expected answers are TGA, TOF, Kawasaki, ALCAPA, VSD with intact IVS, coronary fistulae, coronary collaterals and ruptured sinus of Valsalva. We had a situation in which none of the above featured and yet, our chase for coronaries was essential! It was a 7-month-old child weighing 3 kg with aortic atresia, VSD, normal sized ventricles, large AP window feeding the ascending aorta, type B arch interruption and the PDA continuing into descending aorta! “Why not a truncus” was the logical question. The answer was in coronaries. We could not see proximal aorta at all. The coronaries were visualized, but their origen was elusive. We could not connect the origin of coronaries with the single great vessel that arose from RV. This finding was against the diagnosis of trunk, which by definition should give rise to systemic, pulmonary and coronary circulations. We have asked for a CT scan, but are not really sure if CT can pick up the origin of coronary. The child looked operable on echo. But the plan may be tedious. Translocating the coronaries, routing LV to common vessel, closing the AP window on the pulmonary side, creating RV to PA homograft, correction of type B interruption and PDA closure!! The procedure may take an entire day in the OR for the braveheart surgeon. We are awaiting the cardiac CT report. I shall keep you informed on the progress. Please let us know if any such diagnostic puzzles were encountered involving the coronaries in your experience.

PEDIATRIC CARDIOLOGY PEARLS

36. In pulmonic stenosis, the pulmonary ejection click corresponds to the time when the doming pulmonary valve reaches its open position. The more severe the stenosis, the earlier in systole the click occurs, until it merges with the first heart sound and becomes inaudible. The intensity of the click varies with respiration, decreasing during inspiration and increasing during expiration. These findings enable a confident physical diagnosis. (Vogelpoel L, Schrire V. Circulation journal 1960 page 55)

37. In VSD with Pulmonary atresia, the pulmonary circulation is dependent on PDA or aortopulmonary collaterals. Ductal and collateral sources may coexist in the same patient but only rarely coexist in the same lung. (Liao PK, Edwards WD, Julsrud PR, et al. Journal of American College of Cardiology 1985 page1343)

38. The risk of tetralogy of Fallot increases in the siblings of an affected child. Nora and Nora have suggested sibling recurrence risk of 2.5% if one sibling was affected, and 8% if two or more siblings were affected (Nora JJ, Nora AH. American Journal of Medical Genetics 1988 page 137)

39. In truncus arteriosus, the truncal valve was tricuspid in 277 (69%), quadricuspid in 86 (22%), bicuspid in 35 (9%), pentacuspid in 1 (0.3%), and unicommissural in 1 (0.3%). (Fuglestad S, Puga F, Danielson G. American Journal of Cardiovascular Pathology 1988 page 39)

40. The original classification of Tricuspid Atresia by Kuhne in 1906 was impractical, yet survived as not many were bothered! Once the treatment modalities started, there was a need to refurbish the classification. The present modification of the classification of Tricuspid Atresia as types 1,2 and 3 and subtypes A, B and C was given by Rajesh Tandon and JE Edwards in 1974. (Tandon R, Edwards JE. Journal of Thoracic and Cardiovascular Surgery 1974 page 530)

This brings us to the end of one more post. I am planning to introduce few books of interest for the medical personnel outside their subject. Let me know your take on this plan. Use the comment box for your ideas and suggestions. Use my email id if you find any problem in using the comments box. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed.

Regards

Kiran

2010-06-20T15:43:00.005+05:30

Dr Kiran welcomes everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for everyone with a taste for this phenomenal subject. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. As a part of my interest in medical history, I came across this extraordinary piece of courage and conviction which I felt like sharing. Please go through this and discuss.

Tylenol is the brand name for paracetamol, which is a household name in USA. It is one of the most popular over-the-counter medications and a household name. It was manufactured and marketed by Johnson and Johnson. In contrast to the tablets from of present day, it was available in capsule form 3 decades back.

In 1982, something terrible happened. Eight people who used the Tylenol capsules died. A psychopath claimed that he had put cyanide in some of the capsules. No further information was available as which batches were affected and how many such capsules were likely contaminated.

If you are the boss of a company who has something like 31 million bottles of the drug in the market at that moment with few possible contaminated capsules (claimed and not proven), how would you react?

Random sample testing? Financial assistance to the deceased? Legal battle on proving the non-involvement of the company? Animal testing? Methods to educate the public on ways to find out the non-contamination? Denial? Prove that the deaths are not related to Tylenol? Bribe the officials to get a clean chit? Bury the issue? Some more skeletons in the cabinet? What else?

Mr James Burke, the CEO of J&J at that time was a smart man and a smarter businessman. He could have done anything mentioned above or even more. But what he “did” was amazing.

He opted for full cooperation with the media immediately. He personally appeared on one TV program after another to take responsibility and keep people up to date on the situation. He ordered withdrawal of all the 31 million bottles of Tylenol from the market! He offered to replace the possibly contaminated capsules by Tablets, whose packing was fool-proof. On behalf of the company he accepted responsibility and made it sure to everyone that J&J had not actually done anything wrong, but still is taking the measures for damage control.

It cost J&J a whooping $100 million! More for the replacement in the form of tablets. For the mistake not committed by them.

What J&J gained out of this could not be measured by money. Its way of handling the situation and the decisions taken won applauds from media. "What Johnson & Johnson executives have done is communicate the message that the company is candid, contrite and compassionate, committed to solving the murders and protecting the public," noted the Washington Post.

But what J&J had lost was not just money. It had to recreate the trust. It is not easy when a big thing falls. And Tylenol was the biggest J&J had. It invested heavily in the restoration job. Its share was estimated to be around 37% in the market."It will take time, it will take money, and it will be very difficult; but we consider it a moral imperative, as well as good business, to restore Tylenol to its preeminent position," said James Burke.

It was the responsible handling of the situation that probably saved the day for J&J. It was too big a catastrophe for easy recovery. J&J made it only because its CEO stuck to the ethics, forgetting the money. The Tylenol crisis brought values of J&J into sharp relief. Instead of bringing in a contingency plan, the company carried on by expressing the principles and values. The public could see the transparency of effort, magnitude of the exercise and more importantly, a principled leadership.

Very shortly, public had placed Tylenol on its top position. Its market value enhanced. People could see why they need to trust the brand. Moreover, they were not ready to disown a company which had social responsibility of the magnitude they displayed.

Today, the market share of Tylenol is much larger than what it used to be. Just to end the anecdote, it did not take J&J more than 2 years to recover their financial losses. What they gained out of the display of honesty was priceless.

It takes good principles and strong base of ethics to be a leader. There are bosses so thimble that they cannot even reprimand an undisciplined subordinate! If a problem is brought to their notice, the first thing they do is to demoralize the whistle-blower! They find the whistle-blowers to be a big nuisance. Good or bad, the show must go on with minimal tension for them. Internal nexus with people of cheap morals for ulterior motives by others in the company does not bother them. Honesty need not be honoured; indiscipline need not be punished; cheating someone of their rights need not be corrected – all for own peace of mind. The definition of success in the present day corporate culture depends on how many controversies the leader avoids. After all, avoiding resolving a problem or denying the existence of a problem is much simpler than solving it. Why put your hand into a troublesome beehive when the the affected person is not you?!

Contrasting such scenarios of the present day is what makes James Burke a magnanimous character. He accepted the problem instead of brushing it aside despite his company did not really have anything to do with the actions of a psychopath; the entire thing had happened outside the production and despatch. He instilled a discipline amongst his staff by setting up a crisis management team. He identified the key people who needed to be involved, and limited the number of spokespersons. Most important of all, he took the lead and worked hard in executing the morals. The whistle blowers were honoured. No lazy bum was spared. Profits and losses did not matter in front of ethics. He did not let any internal nexus or outsider encash the situation to their profit. The cash flow would have suffered as the brand was the biggest they had. He accepted it. He sought a lion’s share of trouble by being in the air and media, taking questions, justifying the stand of his company to public and so on. If we can name any one person with maximum loss of sleep and peace in this fiasco, it was the leader, James Burke.

It is just to see what prevails in the end. The loose characters in corporate history who “ruled” with a single objective have perished shortly. It is the people with courage and ethics whose names are remembered today. It is for anyone to choose what they want to be.

Type Tylenol and James Burke in Google. Read the amazing story with many other details when you are free.

With this, let us get back to our regular feature: Interesting leaning scenarios:

DOUBLE LEFT: OK; DOUBLE RIGHT: TOO!

We happened to see a child with a double orifice tricuspid valve. I do not remember to have seen this before. This particular child had one orifice communicating with RV and the other with LV! A large VSD was associated. An ASD had decompressed the RA, so the flow gradient was not found across the TV orifices. Overall, the hemodynamic effects of the lesion were effaced due to coexisting lesions. If such a lesion is seen by you earlier, please let us know how it was and how it behaved.

TOO EARLY OR TOO LATE?

We often come across such situations. We had a 7-month-old first timer who presented with a large PDA. The operability was unclear in the clinical evaluation and echo. On cath, the pulmonary and systemic pressures were identical and Qp/Qs was 0.9:1. Calculated PVRI was 7.3. Is the condition due to failure of primary pressures to fall or rapid development of PVRI? Both possibilities will have opposite ends of management. Should we do something or leave? Would a trial of sildenafil help? Someone suggested a partial ligation of PDA. It will control the Qp in former case if the primary pressures drop. Otherwise, it will act as a pop-off and help the overall survival. It sounded practical, but is it accepted? Please let me know your experiences of handling such situations.

UNACCOUNTABLE

Few children come with diastolic challenges. The picture remains unclear even with the last investigation we can think of. One such had come to us one year back with left ventricular failure. She had a subaortic membrane with moderate gradient across LVOT. Medical management did not get along. She was operated on and the membrane was resected. With a ferocious post-operative battle by team of our intensivists, she could be discharged. She did not come back for follow up for one year and recently, returned with biventricular failure. There was no subaortic membrane this time, but the LVOT gradient was severe. Being refractive to medical therapy, we catheterized her to get a better delineation of anatomy. The EDPs were very high (30-45 mmHg). Her LVOT gradient was 70 mmHg. We projected a high risk and asked surgical team to take a call. They wanted to go in and take the risk of “act of commission”. However, she could not take make it to OT; died a day prior to proposed surgery. The scenario was depressing. We wanted to get a pathological and histochemical diagnosis, but the family was not for it. If such scenarios are seen earlier, please enlighten us on the possible lines of management.

DEVIL AND DEEP BLUE SEA

It is difficult to take a stand in some of the situations. We had a 22-year-old with TOF and diminutive RPA. About 9 years back, he had undergone a BTT shunt. He came back now with history of exercise intolerance and increasing cyanosis. We could not see the BTT shunt on echo. We expected the shunt to have got blocked. On cath, we found a different scene. The RPA had grown a little to about 6 mm. The LPA was big. The left lung had received a good quantity of blood from BTT shunt for the past 9 years and had become hypertensive. The PVRI of left lung was very high. This had lead to the tardiness of BTT shunt. Management was the issue now. Doing nothing Vs doing another palliation. Since the patient was symptomatic, some felt that another shunt should be created to RPA. However, the risk of another procedure, high risk palliation, proposed benefit and other factors took precedence and the patient was decided on medical management. What is the opinion of readership? How would you go about in such cases?

COLOUR FACTOR

An interesting discussion happened between two of the senior consultants of our team recently. The cardiologist quoted a “big man from the west” of having avoided transannular patching in Tetralogies. The statistics and the outcome presented in the data were impressive. However, our surgeon was not impressed. He quoted the anatomical variations of the Hispanic populations versus oriental populations. He said that the Asian anatomy demands transannular patching where as the western may not. These statements open up the possibility for epidemiological research. If anyone knows any further data on this, please let us know.

PEDIATRIC CARDIOLOGY PEARLS

31. In CCTGA, Complete heart block may be as high as 10% at initial presentation and the incidence is cumulative as the age progresses (Bharati S, McCue CM, Tingelstad JB, et al. American Journal of Cardiology 1978 page147)

32. Although the incidence of congenital bicuspid aortic valve is as high as 1.3 to 2% of general population, only 2% of patients with congenitally abnormal aortic valve will experience significant stenosis or regurgitation by adolescence (Bonowro, Carabello B, de Leon AC Jr, et al Journal of American College of Cardiology 1998 page148)

33. Although anterior leaflet of the mitral valve appears to be bigger, it is not so. The posterior mitral leaflet is longer at its base and shorter in its basal-to-apical length than the anterior leaflet. Hence, both the leaflets have approximately the same area (Ranganathan M, Lam JHC, Wigle ED, et al. Circulation journal 1970 page 459)

34. In the mitral valve, congenital cleft is directed anteriorly toward the outflow septum or aortic root in contrast to the cleft in atrioventricular septal defect, which is directed towards the interventricular septum (Smallhorn J, de Leval M, Stark J, et al. British Heart Journal 1982 page 109)

35. A correlation has been found between the anatomy of mitral valve and the central nervous system, more in the behavioural aspects. The subendocardial surface on the atrial aspect of the middle portion of the mitral valve is rich in nerve endings, including afferent nerves; mechanical stimuli from this area caused by abnormal mitral valve coaptation may cause abnormal autonomic nerve feedback between the central nervous system and mitral valve nervous system. Floppy Mitral Valve innervation patterns with distinct nerve terminals provide a neural basis for brain - heart interactions, augmented by mechanical stimuli from the prolapsing Floppy Mitral Valve (Boudoulas H, Schaal SF, Wooley CF. Floppy mitral valve/mitral valve prolapse: Cardiac arrhythmias. In: Vardas PE, ed. Cardiac Arrhythmias, Pacing, and Electrophysiology. London: Kluwer Academic Publishers, 1998: page 95)

This brings us to the end of one more post. I have been answering any comments in the same comment box. So, please go through the comment box if you have posted any idea. I have been sending emails to all the known followers since last couple of times informing about the update. Since we do not have a visitor counter, I will not know of your visit if you do not comment. Use the comment box for your ideas and suggestions. Use my email id if you find any problem in using the comments box. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed.

Regards

Kiran

2010-06-12T23:46:00.001+05:30

This is Dr Kiran welcoming everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for all enthusiastics. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. This small story got reprinted in a prominent daily. It brought out my old memories of fascination when I had read it few years back. Just see how you feel about it!

A couple of decades back in a small town lived a man famous for his intelligence. He was well read and respected. People used to come to him for discussing their problems. He would give them the advice, free of cost. In a way, he was popular as a wise man.

For obvious reasons, certain people in the town disliked him. They wanted to demean him. Since they would not be any match for him on a direct confrontation, they were seeking surrogate opportunities.

One day, someone told the man, “The local politician has been spreading the word that your son is an idiot. Do something”.

The man was surprised. His son was in 4th standard and far from idiocy! He was a clever boy, even though he never topped the class.

The man thought of getting the facts straight. He called his son to chamber and asked, “Do you know the local politician?”

“Yes”, the son answered with a twinkle in his eyes.

“He was saying something bad about you. Any tussle between you?”

“Oh, ignore him, Dad. He is not a smart man”

The man was surprised. The comment was too much for a boy of nine.

“You must not use such terms for elders. I have come to know that the man thinks you are an idiot. But I know that you are not. What makes him think so?”

The boy grinned. “The politician fellow has no good business. He sits with few of his friends everyday in the porch in front of his house. I have to go the same way to reach the school. On seeing, he calls me near him, keeps a 5 rupee coin in one palm and one rupee coin in the other. He asks me to pick up one coin. Once I pick up the coin, he laughs loudly and tells his friends: “Did I not tell you?” asks me to keep the coin for myself. Later all of them laugh again. This happens every day.”

The man was interested. “And what coin do you pick up?”

“Obviously, the one rupee coin”

The man was surprised. “I think you know that 5 is more than one?”

“Of course Dad, I know”

“Still you pick up the one rupee coin?”

The boy looked at his dad in surprise. He excused himself and left the room. He came back within a minute with a glass jar in his hand. The jar was three-fourth full of one-rupee coins.

“What is this?” the man demanded.

“This is to show who the idiot is!” the son laughed.

The man was still unsure whether he understood the situation correctly. “Explain” he told his son.

“It is wits versus stupidity. The moment I pick up the five-rupee coin, this joke would end. The politician has no other business than proving me wrong. I oblige. This will continue as long as I keep picking up the single rupee coin. I want to keep the game alive. Now, I want you to tell me whether 5 rupee coin is worth more or this jar.”

The man was taken aback. His son looked the smartest kid on the world to him at that moment.

The mere feeling that we are better or smarter than others sounds clichéd. The joke is usually on us when we deliberately seek to outsmart others. I recently came across a man who had manipulated the system so effectively that he has his cake and has been eating it too. Only because he makes the people around him feel superior. Every place has such smart people. We often find juniors outsmarting the egoistic seniors, class IV outsmarting their bosses and so on. Whether the act is cheating or outsmarting or manipulating depends on where we stand. If our perspective is of the boy in the story, then we are outsmarting. If it is of the politician, we are getting manipulated! The hunter and the hunted need not be permanent. The places can change. The anecdote is only serves a reminder for better introspection before we attempt to demean the others. Never sure who the victim is!

With this, we shall get back to the regular feature: Interesting clinical scenarios.

SMALL Vs BIG RV

We came across a toddler with TOF physiology with small Tricuspid valve annulus. The resultant RV was suboptimal in size. The decision was for a one-and-a-half ventricle correction. Since pulmonary annulus was small, the decision was to get a transannular patch done. How does such a physiology affect RV and Glenn shunt? Can the free PR be detrimental for RV function? Can the TAP take away the purpose of Glenn shunt in reducing RV preload? Even if RV sustains the load, can the increased preload of RV increase the eventual PA pressures and cause Glenn shunt to fail? If anyone has seen this combo working, please let us know the expected dynamics of post op status.

MASS(IVE) DECISION

A 10-year-old boy was admitted to our ICU with rapidly deteriorating LV function. He came in clear LVF. After the basic resuscitation, we did his echo. The LA and LV were dilated and LV EF was about 20%. Behind the LA, a dense, homogenous mass was visualised. The mass was compressing on the LA, deforming its contours. Moderate MR was seen. One of our colleagues noted a thickened mitral valve with restricted mobility of posterior leaflet. Some pericardial effusion added to confusion. Since the mass did not appear to have much effect on the ventricles and the history was acute, he suspected the diagnosis to be rheumatic, with associated mass. To add on, ASLO titres were positive, ESR and CRP were high. There was no clinical history suggestive of rheumatic fever. Still, the patient was started on steroids in suspicion of indolent rheumatic carditis in failure suggestive of rheumatic activity, along with supportive measures and antibiotics. The patient made a very good recovery on this, with EF increasing to 50% in 48 hours of therapy. We got a CT scan, which showed a homogenous mass behind the heart. The Hounsfield unit measurements favoured the diagnosis of lipoma. With the improvement of EF, repeat echo showed a normal motion of mitral valve, taking away the suspicion of RHD! We have stopped steroids and observing the child, with rest of the supportive treatments on. There are some pertinent questions to be answered. How did a slowly growing lipoma produce the mass effect all of a sudden? If no other super-added disease process is adding on, how do we explain the rapid deterioration in this boy? What post-operative histology can explain all the sequence of events? We are yet to get the surgery done for him due to infection. Once we get further answers, I shall brief you. But the rarity of the case is interesting. Lipomas in the mediastinum are not very frequent. That too, massive ones are very rare. We got just one case report in the literature. If any new waves of thought occur, please post them.

IMMUNE DECISION

How good are the screening tests for primary immune deficiency in a country like India? With such a massive population and rampant consanguinity, our numbers in primary immune deficiency must be enormous. However, no medical student with substantial number of years in paediatrics can recall many such children. Comparatively, our database on HIV is better, thanks to awareness and availability of diagnostic modalities. We had a 9-month-old with vegetations on both AV valves and aortic valve. The child had a significant history of abscess formation with IM injections. Our primary suspicion was immune deficiency. But, primary screening for that turned out to be negative. HIV screening in child and both parents was negative. We involved our hemato-oncologist into the picture, but the scenario did not become any clearer. Assays of immuneglobulins are just one part of the diagnosis. How about the complement function, leukocyte functional assay, receptor level abnormalities and others? Our fellows vehemently chased the diagnosis, but we are financially stressed. We did not want to burden the parents over massive expenditure on investigations. Has anyone come across cases like this anytime? How did you go about the diagnosis? Also of interest is the methodology to be followed in India if a suspected case of primary immune deficiency comes when the immune globulins are of normal levels. Please let us know your viewpoints on it.

UNGUARDED EBSTEINS

What is the DD for Ebsteins anomaly!? The question sounds a bit tough, as there can hardly be any. The picture of Ebsteins is so clear that even partially trained fetal echocardiographers vouch by the diagnosis. We had a baby with such an eventuality. It was reported as Ebsteins from outside. What we found surprised us. It was a case of unguarded tricuspid valve! The leaflets were in the right place with no displacement. But, none of them coapted with each other, leaving an unguarded opening to allow a free to-and-fro movement of the blood. The RA was enlarged and the moderator band looked thick and mobile, giving an impression of displaced STL. Is this condition common? It is possible to have missed this condition for Ebstiens. Please let me know if you have seen such entity earlier.

AGELESS REGURGITATION!

How would the pulmonary regurgitation behave with age in setting of absent pulmonic valve? We had a 24-year-old with TOF and absent pulmonic valve! His PR was minimal, but the PAs were huge. The latter findings suggest that the PR must have been there and significant. How to explain the minimal PR that we find now? No one could recall any patient of this age with such findings. Does the free/severe PR improve with age in absent pulmonic valve syndrome? If anyone has come across such a scenario, please let us know.

PEDIATRIC CARDIOLOGY PEARLS:

26. There is a sub-variant of Partial AV canal defect called Transitional type. In the transitional form of partial AVSD, there is aneurysmal replacement of a portion of the inlet ventricular septum, leaving a minimal VSD with a tricuspid pouch which usually obstructs any major shunting at the ventricular level. (Seward JB, Tajik AJ, Edwards WD, et al. Congenital heart disease. In: Two-Dimensional Echocardiographic Atlas. Vol. 1. New York: Springer-Verlag, 1987)

27. In 1973, when echocardiographic evaluation was not a part of diagnosis, it was suggested that the diameter of the ventricular septal defect compared to the diameter of the ascending aorta could be used in determining the prognosis. When the ratio was ≥ 0.8, this defect was considered large, was unlikely to spontaneously close, and the infant would have a large pulmonary blood flow and pulmonary artery hypertension. Although the determinants are changed with the advents of Echocardiography, few still consider this point as a significant one. (Rowe RD. In: Barratt- Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy. Diagnosis and Surgical Treatment. Proceedings of the Second International Symposium. Edinburgh: Churchill Livingstone, 1973 page 121)

28. In Atrial Septal defect, Eisenmenger reaction was found to be 7% in the first decade, 8% in the second decade, 10% in the third decade, and 11% in the fourth decade and beyond. (Cherian G, Uthaman CB, Durairaj M et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. American Heart Journal 1983 page 954)

29. The commonest congenital heart disease with right aortic arch is common arterial trunk (popularly called Truncus arteriosus) (Anderson RH, Thiene G. European Journal of Cardiothorac Surgery 1989 page 481)

30. In d-TGA newborns, increased numbers and size of pancreatic islet cells, and increased weight of the adrenal cortex are found. These findings in the pancreas and adrenal cortex are similar to those seen in infants of diabetic mothers and support the contention that the higher-than-usual glucose concentration in the descending aorta during fetal development may play a role (Naeye RL. Archives of Pathology 1966 page 412)

This brings us to the end of one more post. I had sent the emails to all the known followers last time. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed. Also, send your feedbacks by email or via the comments section.

Regards

KIran

2010-06-04T23:18:00.000+05:30

Welcome to the readership. This is Dr Kiran inviting you to another post of blog. As previous, we shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, as we have been doing since past posts, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. The following story was told by one of the motivational speakers in a seminar. The speaker was author of multiple best-sellers and does motivational courses nowadays. Please go through this interesting anecdote.

A mother came with her adolescent son to a wise man. It was clear that the boy was brought there by emotional force! He looked uninterested.

“Sir”, the mother addressed the wise man. “This is my son. He is very intelligent, but not focused in life. I request you to give some advice to him”.

The wise man smiled at the boy, who frowned impatiently.

The wise man was indeed wise. He was a modern day Guru. He would not preach or advice atonement or punishments. His words were always more practical than many would think.

“Can we talk about something?” the wise man invited the boy into conversation.

The boy thought of leaving. He looked out of the window. A freshly asphalted road was seen. A small ball of tar was lying at a corner.

“Let us talk about that small ball of tar” the boy said, trying to ridicule the wise man.

The wise man did not get offended. “Can you please get that ball of tar?” he asked.

The boy felt happy. He wanted the “wise man” to give back nicely. He fetched the tar ball and gave it.

“How much do you think this tar ball would cost?” the wise man asked the boy.

“Cost? It is useless; worthless” the boy replied.

“These are basic hydrocarbaons, aren’t they? How much do you think its raw material would have costed?” the wise man had a conviction in his words this time.

“Boy, this fellow knows some chemistry too” the boy thought. He wanted to show off now. “May be, about ten bucks” he answered cautiously.

“Hmmm. Let us get few steps back. This is made of the same basic chemicals used to make plastics I believe?”

“Yes”, the boy was enthusiastic this time. “Vinyls and other polymers would have the basic molecular skeleton as this”

“High quality polymers are expensive. Is the same basic chemical composition applied there also?”

“That is correct. The hydrocarbons have same basic chemical structure. They can be sent through different pathways to obtain different substances. But the basic chemical skeleton remains the same”, the boy was feeling proud this time.

“I hear some of the finest and highest quality materials used in rockets and medical instruments can be prepared using the same chemical framework. I was told by a scientist that these substances cost almost Rs 10,000/- per meter” the wise man said with an innocent tone in his voice.

“You have heard him right. These are very high quality substances that need to be segregated at a very early stage of hydrocarbon processing. The channelization actually matters. Of course, high level of discipline and proper instrumentation along with appropriate environmental standards make all the difference”, the boy knew his stuff and was eager to show off.

“I was told mere ten grams of the basic substance can make high quality thread as long as 1,000 meters. Can it?”

“Of course. Such material used in critical processes may cost about Rs 1,000 a meter” the boy said with full vigor in his voice.

“So, you mean to say this useless, worthless ball of tar could have been something worth a million rupees only if were to get channelized at the beginning”, the voice of wise man was impassionate.

Silence. The boy did not say anything.

“Don’t you think the same process gets reflected in life?” the wise man saw into the eyes of the boy.

The boy bent his head. Words were struck in his throat. “Yes” he said slowly.

“Power, knowledge and intelligence may not reach where it deserves unless it gets a sense of direction and channelized into the proper pathway. It is left to you whether you wish to end up Rs1000 a meter or a waste mass of material at a deserted corner of road”, the wise man stopped.

The boy choked. The message was conveyed.

I remember one of my physics lecturers telling the class. “Be a vector. Your energies are nothing without the element of direction.” Not many of us could understand or appreciate what he told at that age. However, the words remained with me. I see a greater meaning now for the same. Every day, the same message gets extrapolated in many fronts. Even when it comes to management of heart problems, we often see how the small steps kept at early phases of disease process end up being great benefits for the child. Similarly, how small mistakes at the inception end up being a disaster for the patient. Appropriate channelization, whether in life or in any trifle looking setting, is indeed a big task. I felt like sharing this anecdote for the final punch it delivers and the resultant impact!

With this, it is time to get back to our regular feature: Interesting case scenarios:

INEVITABLE LAST MINUTE

We had 8-year-old with single ventricle physiology with IVC interruption and pulmonary atresia and intact IAS. How would the cath for single ventricle suitability help? We cannot enter the PA, cannot get the pulmonary venous wedge or have any way of measuring the PA pressures. Since it is a potential Kawashima, unless the data is clear, the decisions are pending. We contemplated this, yet went on with cath. Cath study did not spring any surprises and no clear data could be obtained. It was left to “on the table” decision making. Is there any other way of getting the data from cath? Please let me know your opinions on such scenarios.

POSSIBLY POSSIBLE!!

Is there a possibility of transposition of great arteries with {S,D,S} combination? In other words, can we have normally related great arteries in transposition diagnosis? The words sound paradoxical. However, we came across such a report done from outside. On repeating the echo, we found a DORV, NRGA, VSD, severe PAH. The commitment of PA was unclear and could be routed to RV or LV depending on how the patch is to be directed. When we put up the question on the possibility of outside report, we were surprised to hear that {S,D,S} in TGA is possible and reported. Has anyone come across this combination? If so, please send the details. We shall put it up in the subsequent posts.

CALCIUM DYNAMICS: IS PREDICTION POSSIBLE?

We had one 11-year-old girl, who had undergone RV to PA homograft conduit repair about 11 months back. The girl came back with calcification of homograft with severe obstruction. On the cath study, we found calcification on the VSD patch also! The surprise element was the rapidity with which the calcification had occurred and progressed. The surgical team introspected about the type of valve. They wanted to see the records to find out if it was an antibiotic prepared valve or a cryo preserved valve. Does it actually matter? Is there any way in which we can predict the calcification? Are there any observations regarding these issues? Please let me know your experiences on this.

NUMERICAL DILEMMAS

Sometimes, the initial assessment of a patient with high Qp and sever PAH shows clear operability on clinical assessment and basic investigations. However, the cath data on the same patient may not show the same picture. We had one 8-year-old of CCTGA, VSD with PAH. He was saturating 98%. However, the surgical team sought a cath study to ensure the anatomy. On the cath, the PVRI was 14.8 wood units! The surgery was ruled out due to the numbers. Many of us would have come across such situations. Is there a cut off for ordering additional investigations? If there is a disparity between the numerical values and clinical findings, doesn’t the latter get precedence? How to resolve such issues? If you have found any solution for these scenarios, please let us know.

COLLATERAL DAMAGE

We come across scenarios wherein the patients with single ventricle physiologies saturating well would be put on Glenn shunt for reducing the volume loads. We had a 2-year-old with single pump physiology saturating 92% in room air. He had good aortopulmonary collaterals. How should we go about dealing with these collaterals after the Glenn shunt? Do the saturations come down if we ligate the collaterals? Is a low SO2 after surgery acceptable? If we leave the collaterals, is the risk of overflow and volume load higher? The senior surgeons always maintain that the collaterals are self limiting and if jobless, they would involute by themselves. Is that true? If so, how long do they take? Is accepting low saturations better than risking overflow and volume load? The surgeons were reluctant due to issues related to approach. They suggested that we can coil the collaterals post op. What is more logical? If any other institute has any consensus on this issue, we would be interested to know their logic.

PEDIATRIC CARDIOLOGY PEARLS:

21. Arterial malformations have two vascular patterns: Arterio Venous Malformations (AVM) and Arterio Venous Fistulae (AVF). AVM (microfistulas) are multiple arterial feeders joined via a nidus to draining veins. AVF (macrofistulas) are direct shunts between large arterial and venous channels. (Avery JB, ed. Cardiovascular Pathology in Infants and Children. Philadelphia: WB Saunders, 1984 page277)

22. The relationship between development of aortic arch and migration of neural crest cells into the pharyngeal arches was given for first time in 1991 in avian embryo studies. (Kuratani S, Kirby ML. American Journal of Anatomy 1991 page 215)

23. Normally, there are two right and two left pulmonary veins. The most common variation is the presence of a single pulmonary vein on either the right or left side, with a prevalence of about 24% in anatomic studies. Contrary to the popular belief, the prevalence of a third pulmonary vein on either the right or left side is only 1.6% to 2%. (Healy JE Jr. Journal of Thoracic and Cardiovascular Surgery 1952 page 433)

24. The persistent Left SVC opens into coronary sinus and drains into Right atrium. Hence, the coronary sinus enlarges in presence of left SVC. This interferes with blood flow from the left atrium into the left ventricle. An increase in the magnitude of the left-to-right shunt at the atrial level was found in patients with secundum atrial septal defects (ASDs), persistent LSVC, and dilated coronary sinus. (Byerregaard P, Laursen HB. Acta Paediatrica Scandinevia 1980 page 105)

25. Left axis deviation in ECG is found in moderate VSD. However, large VSDs and equal ventricular pressures demonstrate right ventricular hypertrophy pattern in ECG. (van den Heuvel F, Timmers T, Hess J. British Heart Journal 1995 page 49)

This brings us to the end of one more post. I had sent the emails to all the known followers last time. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed. Also, send your feedbacks by email or via the comments section.

Regards

KIran

2010-05-15T22:54:00.000+05:30

Hello everyone. This is Dr Kiran welcoming you to another post of blog. We shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. This particular anecdote was put up in newspaper article. I happened to call the author and found it to be semiautobiographical. I found this worth sharing. Please go through.

Rakesh was 18 years old. He was never much interested in studies. He always had an ear for music, although he was a lousy singer by himself. He would attend many concerts at his place whenever he found an opportunity, setting everything else aside.

Rakesh’s father owned a small departmental store. They were not super-rich, but were well to do. Father’s ambition was to see a future for his son. As the son was never interested in future studies and had a minimal chances for an office job, his father had intentions of putting him into their business. However, Rakesh never showed any interest in business.

Rakesh had many friends who were equally interested in music. They used to attend the concerts together. Most of them were from middle class families and were not as well to do as Rakesh.

One day the father found Rakesh sitting alone in the living room. “What’s the matter?”, he enquired.

“Nothing great”, replied Rakesh and showed a newspaper item to his dad.

It was about a music concert at a place about 1000 km from their place.

“This man is great. I wish I could have attended his concert”, said Rakesh in a low tone.

His father saw the newspaper item for few moments. “Mind if I join you for the concert?” he asked his son.

Rakesh could not believe his ears. “Really? By all means” he said. “My accommodation problem also solved!” he said silently to himself.

They had a great journey by flight; stayed in a good lodge. They attended the concert. Rakesh was thrilled. His father just sat next to Rakesh throughout the concert.

They were back in the lodge and started packing. They were due for departure early morning.

“I don’t know how to thank you, Dad”, said Rakesh. “I never thought you enjoyed music. All my friends wanted to attend this concert, but you made it for me.”

“Two things” his father said. “First: I don’t enjoy music. But I do enjoy you enjoying it. I wanted to see you happy. So, I joined you all this distance. I am happy that you are happy.”

Rakesh was stunned. His father had driven the point quite well.

“Second”, his father continued. “You said lot of your friends wanted to attend this concert. None could. Largely because they could not afford the travel, stay and all the other expenses.”

Rakesh listened attentively. His father continued.

“In life, both the things should be balanced. One should have a passion: music as in your case. Also, everyone should have a profession which can make the passion possible and reachable. I could make your passion possible because I have a profession which can afford it. Lack of the second would make the first redundant. That is what has happened to your friends.”

Rakesh could appreciate what his father said. His father continued.

“I do not specify the profession. It can be anything of your choice. Government job, private firms, business, contract, whatever it may be. One should have an income enough to sustain the needs of himself and his dependents. At the same time, the needs should not extend the stretchable limits of income. This is the balance one has to achieve. If you wanted to attend a concert somewhere abroad, I probably would not have afforded.”

Rakesh appreciated the genuine tone of his father’s words. He did not feel any preaching. His father sounded more practical than he ever had been.

“In your case, I can give you an option to take care of our store. It is purely your wish. If you want to continue to study, please do so. If you want to find an office job, try it. But, don’t stay stale. Keep doing something constructive or potentially constructive. Early birds usually have an advantage.”

Rakesh wanted to listen, but his dad stopped there. There was some silence. Without many words, they completed packing.

They returned home. That evening Rakesh reached their departmental store. He waited till his dad completed a transaction with a customer. “Yes, Rakesh”, his father asked.

“Dad”, Rakesh replied. “Can you teach me how our store functions? I want to join as a regular salesperson and learn the trade.”

Tears of joy filled the eyes of his father.

If there is anything that we in medical profession miss, then it is the passion for non-medical issues. I have rarely seen medical professionals who have kept the other passions intact. I had heard the story of a famous neurosurgeon who cried on the day of his retirement for not having continued his passion for violin. Also, I knew a medical student who tried to seriously pursue his passion for painting along with medicine and failed in both. It is preferable to have a mature person advising us on how to balance both, but not everyone may find one. This story may tell us something we have already learnt many a times. Yet, it may be worth the pondering.

With this, we shall get back to the interesting learning scenarios.

ALTERNATE PATHWAY

In scientific temper, it is very difficult to accept something without adequately exploring all the possibilities. We had a 3-month-old with Tricuspid atresia 1A. There was only a restrictive PDA. However, the pulmonary venous return was much more impressive than what was suggested by PDA. But on echo, we could not locate any other shunt. A continuous murmur suggested the presence of collaterals, but the same could not be visualised on echo. Since the saturations were less, it was decided to go for a BT shunt. On the table, a coronary fistula was found to distal PA! This explained the picture clearly. Sometimes there is more to the patient than what meets the echo eyes! Please let me know if you have seen similar pictures.

ALL OR NONE?!

We had a 4-year-old with TGA, multiple VSDs, moderate to severe PS with L-malposed great arteries, saturating 85% in room air. On cath, the data appeared to be suitable for ASO with Rastelli repair. The additional VSDs were an issue. The surgical team felt that as many VSDs should be closed as possible. Even then, few VSDs may remain and the final procedure would be a palliative ASO. We were thinking whether such a heroic procedure is worth it. If some VSDs remain, they themselves may be a major cause for concern. Unless we can ensure complete closure of all VSDs, ASO in L-malposed set up may not be worth all the high risk procedure. With the balanced physiology the patient had, one of the contemplations was to leave him alone. What is the opinion of the readership? Let us know your take on this.

RISK Vs RISKIER

We often face problems in those age groups that we are very comfortable with. We had a 21-year-old with cTGA, VSD, PS in the setting of situs inversus. He was saturating 87% in room air. Cath data showed a routable VSD. But his ventricular EDP was 20 mmHg. It was presumed that the raised EDP was secondary to chronic hypoxia and cyanosis. One option was to do a double switch – Senning with Rastelli. The caveat was about the success of Senning in high EDPs. The other option was to leave him as he is now. This may lead to further dysfunction of ventricles with time. Is the option of doing the surgery in situs inversus and its outcome riskier than the medical follow up? What is the natural history of untreated cTGA, VSD, PS? What the average progress of the ventricular dysfunction in such cases? Is there an index for predicting the progress? Such questions remain unanswered. Neither the surgical team nor our team could find an answer for these questions. If anyone has any experiences in these issues, please let us know.

DOUBLE JEOPERDY

One of the inherent weaknesses of the human mind is to fit the findings to a diagnosis that is known! Sometimes, when all the findings are somehow not fitting into a clear picture, we presume that it is a variant of a known diagnosis and try to fit it. Same happened to me one of these days. We had a 9-year-old coming to us with left upper limb hypertension and low BP on right along with absent femorals. I could see a right arch which was narrow at isthmus and the adjacent subclavian showing turbulence at the origin. Since it was a right arch and the echo windows were not to boast of, I presumed a mirror imaging and gave a diagnosis of possible Takayasu disease. However, the cardiac CT undid the actual diagnosis. It was right arch with normal branching, coarctation of aorta at isthmus with aberrant origin of right subclavian artery from the post stenotic segment with the diverticulum of Kommarel. The combination could easily explain the clinical picture. With 2 vessels showing the turbulence with the disparity of pulses, I was lured into making a diagnosis of Takayasu disease neglecting all other markers!! In retrospect, I feel that if I had spent some more time in visualising the arch, I would probably had better chances of making the correct diagnosis. It was a lesson re-learnt. I don’t know how long it will stay! Let me know if you were also lured into making any such diagnosis which caused certain regret on retrospect.

SHUNT IN REGURGITATION

This issue has always troubled me, but did not have a platform to discuss. I am presenting this before everyone for the individual opinion. If anyone happens to find a literature evidence, please support it. The question is: What happens to Qp/Qs in cases of VSD with MR? We had a 6-year-old with complete AV canal defect saturating 92%. He had severe AV valve regurgitation. His calculated Qp/Qs was 1.15:1. Based on the number, he was considered as inoperable. The question was: if there were to be no AVVR, would his Qp/Qs be different? Doesn’t VSD get underestimated in the presence of AVVR? Since AVVR is PVRI independent, the VSD shunt would definitely be influenced by AVVR. Since the AVVR is surgically correctable in this case, the logic of refusing the surgery based on original Qp/Qs correct? The original question still remains. Is the Qp/Qs affected by MR? Or, is the regurgitant volume remains the same and gets nullified in the final equation? Please enlighten.

PEDIATRIC CARDIOLOGY PEARLS:

16. The recommended age for the closure of ASD is around 4 years. This was based on a study in 1983 by Cocherham et al in 87 children with ASD. It is followed as a matter of fact in all places. (Cockerham JT, Martin TC, Gutierrez FR, et al. American Journal of Cardiology 1983 page 1267)

17. An interesting term called double outlet right atrium was introduced by Horiuchi et al in 1976. It involves a primum defect with deviation of interatrial septum to the left. In true sense, it is only AV Canal defect. (Horiuchi T, Saji K, Osuka Y, et al. Journal of Cardiovascular Surgery (Torino) 1976 page 157)

18. Patients with outlet ventricular septal defects usually have deficiency of muscular or fibrous support below the aortic valve with herniation of the right coronary leaflet through the VSD. This leads to progressive aortic valve regurgitation with time. Hence, it is important to recognise this at the earliest and do a surgical correction to avoid any damage to aortic valve. (Van Praagh R, McNamara JJ. American Heart Journal 1968 page 604)

19. The continuous murmur of PDA has the highest number of descriptive names! It was originally described by Gibson in 1900 as having late systolic accentuation and continuation through the second sound into diastole. He, incidentally, never used the term continuous! (Gibson GA. Persistence of the arterial duct and its diagnosis. Edinburgh Medical Journal 1900 page 1)

20. Bland White Garland syndrome refers to ALCAPA. It goes by the names of 3 physicians who described it in 1933. Few have confused it for a bland patient, who looks white because of ischemia and ready for a garland on passing away! (Bland EF, White PD, Garland J. American Heart Journal 1933 page 787)

This brings us to the end of another post. My good friend Dr Prem Alva suggested informing all the followers by email on every update. Sounds practical. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed. Also, send your feedbacks by email or via the comments section.

Regards

KIran

2010-05-09T00:06:00.001+05:30

Hello all. This is Dr Kiran, welcoming you to the present session of blog. We were in the process of learning greater meaning of few anecdotes. The present anecdote was picked up from a newspaper article. Please go through.

Mr Iyer was a busy man. He worked for a MNC. Clock and calendars did not have any role in his life. He would often work for days together without coming home. He was known for his rigid stands, no-nonsense approach and upright decisions. When some lesser mortals made the mistake of asking about his salary, he would reply, “Rs.1000 per hour” to prevent them from talking any further.

Mr Iyer had a relatively big test the next day. He was supposed to give a business presentation to one of the global biggies. True to his meticulous self, he had done everything to perfection. He wanted to make sure that the things are alright. He took the previous day off and stayed home to fine-tune his presentation.

Mr Iyer’s son was a bubbly 5-year-old. People described him of having inherited his father’s intelligence. The son was elated to see his dad home that day and refused to go to school.

Mr Iyer did not heed much importance to this issue. He wanted peace of mind. He thought that losing his temper on anything may affect his performance the next day. He just let his son stay back.

The scene was not as simple as Mr Iyer thought. His son came down to the dad every two minutes and kept talking to him. Mr Iyer tried his best to calm himself, but his son was too insisting.

“Don’t you see that I am working on a presentation? This is important. Tell me what you want and let go of me” he tried to negotiate from his son.

The son was shocked for a while. He left the room, but returned 5 minutes later. “Dad”, he cried. “Can I have Rs 100/- from you please?”

On other times, Mr Iyer would have asked for reason, but this day was unlike that. He brought out his wallet and threw a Rs 100/- bill on his son. “Take this and leave” he demanded.

The boy picked up the cash from the floor and left. He returned in next five minutes. “Dad”, he said softly this time.

Mr Iyer’s temper was rising. He was getting annoyed. He grinded his teeth and asked in a stern voice, “What now?” “Please see” the son demanded.

Mr Iyer decided that unless some force is applied, this disturbance would persist. He got up from his chair and was about to thrash his son, when he noticed the clutched fist of his son held towards him.

“What is in your hand?” Mr Iyer asked angrily.

His son slowly unclenched his fist. There were few notes of various designations in the hand.

Mr Iyer was perplexed. “What are you doing? What do you want?” he asked in the same tone of annoyance.

“I opened my piggy bank. It had Rs 400/- I have taken Rs 100/- from you.”

“So?” the tone of Mr Iyer was mellowing down.

“I have heard you telling many uncles that you earn Rs 1000/- per hour.”

Mr Iyer was silent this time. His son continued.

“I have Rs 500/- with me. Can you please take this and play with me for half an hour?”

Mr Iyer went back to his laptop and shut it down. He played and spent time with his son the rest of the day. Mr Iyer was so much pleased with himself that his presentation the next day was applauded by everyone as the best he had done till then.

Very often, we do not understand the value small things. What we perceive as a small issue may really be a big one for the others. Our few minutes may be what the opposite person desperately needs. It is true with our family members and patients too. Often, big-wigs of the hospitals find it futile to find any time for their subordinates and patient attendants. I have seen busy practitioners starting their day around 5 pm and going home back by midnight. They are proud of their time management skills and claim that they stretch a couple of hours sleep in their cars during the travel. Few also say that their kids would not have woken up when they leave the house and would have slept well by the time they reach home. One of our consultants was very angry that he had to apply leave for a day to attend his son’s school day, as the boy was insisting on it a lot. It is very common in medical profession to have such scenarios. What majority does usually become a rule and makes an easy way others to follow. Doctors who try to defy the rule are termed “lazy”, “useless”, “waste body” and so on. It may be prudent to understand what the time management actually stands for before deciding on to invest on it. This small anecdote can open up lot of these issues pertinent to each of us in its own way.

With this, let us go back to the interesting learning scenarios of this post.

PROBLEM WITH WINDOWS

We had a situation which took lot of cerebral exercise. This 11-year-old boy had undergone closure of AP Window and PDA few years back. He came back to us with exertional dyspneoa. On echo, we reported him as possible residual PDA as suggested by the location of jet. However, on cath study, the outcome was a residual AP window. The data showed operability on oxygen study. During the cath meeting, our senior surgeon had a different opinion. He felt that the cause of this residual APW after these many years might be due to high PVRI per se and the residual opening is actually a pop-off. In his opinion, such lesions should be left alone if the symptoms are not much pressing. But the data we had did not suggest the same. Hence, we tried to attempt device closure of the lesion. We initially tried a VSD device, but it slipped. After few days, we tried a PDA device unsuccessfully. Now, we may not attempt the non-surgical ways anymore. What is the opinion of the readership on this? We shall keep you informed on the progress of this boy. Let me know your ideas on this issue.

PULMONARY CONTRIBUTION

We often see Qp/Qs in cath studies that are too high to believe. Yet, the pressure from dilated pulmonary arteries on the bronchi is not too high to compress the respiratory tract. We had a 8-month-old blue boy with large ASD shunting bidirectional and multiple muscular VSDs shunting right to left. The CHD lesions could not explain the clinical scenario. We found reduced air entry on the left lung. The chest radiograph showed collapsed left lung. The cardiac CT showed a completely collapsed left bronchus with collapsed left lung and a dilated left pulmonary artery adjacent to the bronchus. How to chronalize the cause-effect relationship? The surgical team felt that the dilated PA must have compressed the Left bronchus and the resultant lung collapse must have contributed to the PVRI causing right to left shunt. But, haven’t we seen ample number of large ASDs? How many times do we recall such a picture happening? Our logic was different. There might be an inherent defect in the left bronchus, which might have collapsed early due to added pressure by the LPA. Who is correct? We asked for pediatric surgical opinion for which a bronchoscopy for learning about the status of left bronchus. But the bronchoscopy was not much helpful. We were not clear on how to proceed. I hope the readership can also participate in this cerebral exercise for a while and come out with their suggestion.

BALLOON DECISIONS

How far is the balloon occlusion helpful in decision making of PDA operability? The technique or the interpretation does not seem to be standardized. We had an adult lady with a decent PDA shunting left to right. The balloon occlusion data was not helpful either way. We still went ahead with the device closure based on clinical and echo data. It turned out to be successful. The patient went home well. If we had gone by the cath data alone, we probably would have not touched the patient. Taking the entire picture, the cath data on balloon occlusion was invalid. Can the readership inform any published data on the proper balloon occlusion technique and interpretation? Please let me know.

TRACING THE PROBLEM

It was a nice question which we never had thought prior. The LV and RV tracings in the cath are very characteristic and reproducible. What causes the difference in the morphology of the tracing pattern? The question came up when one of the students put up this question in an e-class. “What does the LV tracing look like in a TGA?” Logical answer may be “like an RV tracing”. But, what is the correct answer? Please let me know the answer with references.

DISGUISING AS TAPVC

We had a 2-month-old who came to us with an echo report from outside as TAPVC. On the echo, we were surprised with the left to right shunting across the ASD. The left atrium looked very small with a normal mitral valve. The pulmonary veins were seen draining into the left atrium. Why was the LA small? On a close look, we found a bleak line within the presumed LA outside which no colour percolated. We decided that the echo free space is likely to be a cyst. Cardiac CT defined the mass to be cystic. On the surgical table, the cystic lesion was confirmed. There was no TAPVC. The smallish LA was secondary to an external compression by a cystic mass. It was only because of a machine with good resolution that we could pick up the diagnosis. I wonder what I could have done with a suboptimal machine that I am usually handed with at peripheral centres! If the readership has come across any similar events, please let us know.

PEDIATRIC CARDIOLOGY PEARLS:

11. The embrtological sequence of atrial septation is one of the most interesting understandings for both students and examiners. This hypothesis has been successful in explaining the abnormalities of atrial sepatations. This sequence of events was explained for the first time by van Mierop in 1976. Even today, we follow the same with few minor modifications. (Van Mierop LHS. In: Feldt RH, McGoon DC, Ongley PA, et al., eds. Atrioventricular Canal Defects. WB Saunders publications, 1976: page 12)

12. Children with Down syndrome are more likely to have complete AVSD than children without Down syndrome. They are also more likely to have associated tetralogy of Fallot (Vet TW, Ottenkamp. J in American Journal of Diseases in Children 1989, page 1362)

13. VSD happens to have most variants of classification. The most accepted version was published by Soto et al in 1980. In this classification, the ventricular septum is considered to have four components: An inlet septum separating the mitral and tricuspid valves; a trabecular septum, which extends from the attachments of the tricuspid leaflets outward to the apex and upward to the crista supraventricularis; the smooth-walled outlet or infundibular septum, which extends from the crista to the pulmonary valve; and the membranous septum, which is relatively small and is usually divided into two parts by the septal leaflet of the tricuspid valve. Each zone has defects going by their generic name. (Soto B, Becker AE, Moulaert AJ, et al, British Heart Journal 1980, page 33)

14. The incidence of PDA is about 30 times greater at high altitude (4,500 to 5,000 m) than at sea level (Alzamora-Castro V, Battilana G, Abugattas R, et al. American Journal of Cardiology 1960, page 761)

15. In Tetralogy of Fallot, about 40% of patients have an abnormally long, large conus artery from the right coronary artery that supplies a significant mass of myocardium. In 4% to 5%, the left anterior descending coronary artery arises from the right coronary artery and passes across the right ventricular outflow tract (RVOT). The resultant abnormal anatomy leads to compromises for surgical repair in the region of RVOT and pulmonary annulus. Hence, if the echo does not pick up the anomaly, one needs cath for proper surgical planning. (Fellows KE, Freed MD, Keane JF, et al. Circulation journal 1975, page 561)

With this, the present post ends. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am planning to add few more things to the new segment. Please ask your general pediatric friends about the blog and get their feedback on what would their need be.

Regards

Kiran

2010-04-17T20:09:00.000+05:30

Welcome to NH Blog. This is Dr Kiran. We have been exploring the wonderful world of pediatric cardiology for past few months. As previous, we shall continue with small anecdotes that convey more meanings.

The following story reached me via internet. One of the followers of this blog asked me to share this others. Due to credit to him, please go through this wonderful anecdote.

“How many times have you appreciated someone for no reason?” asked a renowned speaker in an annual meeting of a big company.

Eyebrows rose. “Why should we?” questioned the minds of top brass. “They have no eyes to appreciate the good, forget the same for no reason” mumbled the lower rung.

“Now think honestly for yourself; how many times you really felt like appreciating someone for their good work and did not do it for reasons that need not be disclosed?”

The hall went silent. The introspection was evident.

“After sometime”, continued the speaker, “it is very easy to forget the good job and the person who did it. Even if we try hard, we may not recall both the person and work. As the human nature goes, we forget the entire episode very easily.”

“So”, cried a voice from the middle of the crowd.

“Don’t postpone” said the wise man. “You have lost lot of opportunities to sincerely represent your feelings. Now, you can atone for it by appreciating someone who has done service to you. Let it be for no reason. Let it be a surprise for them. Let it come from nowhere for them. Try it out. Both of you will really feel better” he concluded.

The CEO of the firm thanked the speaker in his vote of thanks. "Our distinguished speaker made an exemplary point in his speech. We have been working on traditional moulds and outsets which preach that our relationships must be all business, cognitive, stand-offish and nonemotional. The most powerful thing he has taught us today is to inculcate a corporate culture with emotional human connection. From now on, I request all of you to freely express your appreciation to each other. This stuff could be contagious for a better world. Pass it on!"

When the function got over, the CEO was still haunted by the happenings of the day. On his way back home, he thanked his driver. "Thanks," he said. "For four years you have been my company driver and I don't think I have ever told you how wonderful you are. You are the best driver on the road. You are a first-class gentleman and an asset to our organization. I need you and deeply appreciate your friendship and loyalty."

The chauffeur was ecstatic. The CEO of the firm saying all the good things to him looked unbelievable. He was whistling and smiling like never before when he reached home. He went to his wife, hugged her and told, “I am feeling great today. I feel I have really achieved something. Lots of it is because of you. You are such a support and love. I don’t think I would have been the same without you. Thank you. Let’s go out for dinner tonight.”

The couple went to a nearby restaurant which was a tad expensive for them, but the man was too happy to afford that the day. Their son, who was sent out of the house for some reason a year back, worked there. The son didn't know what to say when he saw the parents. His father did something he could not believe. The father walked straight over to his son and hugged him. With tears in his eyes he said, "I love you, son. I appreciate you for what you are. Please come home. I miss you and need a relationship with you."

The boy went nervous. He had never seen his father like this. He excused himself from his parents and went into the manger’s chamber. "I've decided not to quit this job, sir. I have decide not to move out of this town," he told the manager. "I've just decided to move back in with my parents.” “I always thought your father was a strict disciplinarian. He never approved the free lifestyle you wanted to have. The boys tell me that you were sent out of the house when you got your body tattooed and changed your hair colour to join the music band. Why is the sudden change of decision?" the manager asked. "I just discovered my parents actually care about me and I'm sick of letting them down trying to prove my independence. It's time to make them proud!"

The story opens up a lot of things. There are lot of ways this can be interpreted. A genuine expression of appreciation is contagious and leads to good cascade. It is just the story of one person who followed it. The others who followed it might have had similar effects. The corporate culture has to break away from the mould. But, one word of warning. People with lesser maturity may not appreciate the difference between independence and indiscipline. So, even when a word of appreciation is handed over, the recipient should be really worth it. Try it out. It may be worth it.

With this, let us move on to our learning scenarios segment:

LOCATION: MATTERS?

Does the location of VSD matter for the velocity of the development of PVRI? In one of our meetings it was mentioned that between perimembranous VSD and subpulmonic VSD of same size, the latter develops early PVRI. I could not find any reference for the same. It can be logically concluded that the perimembranous VSDs have a chance of developing RVOT muscle bundles, but the subpulmonics would not. Also, the closer the VSD to the pulmonic valve, higher the chances of them behaving like aortopulmonary shunts. Does this theory find any takers? Are there any studies or observations to prove them? If similar findings are observed by you, please let me know.

JUXTAPOSED TGA

How common is it to find juxtaposed atrial appendage in TGA? We had a 2-month-old with TGA, intact IVS with juxtaposed atrial appendages. We had no recall of the last time we had this combination. Also, this baby had a smallish RPA with a normal sized LPA. How would one go about treating this combination? Our surgical team felt that a Senning would not be possible due to juxtaposition. The age and anatomy were not in favour of arterial switch. But, how to explain the disparity in the sizes of PAs? Has the appendage juxtaposition got anything to do with branch PA anatomy? Are they related in any way? Please let me know your ideas on it.

DISCONTINUITY

One of the major criteria for diagnosis of DORV is mitral-aortic discontinuity. When the great arteries are malposed, it would be mitral-pulmonic discontinuity. Does the same criteria hold good for DOLV too? Recently, we had a 6-month-old with this picture. AV concordance was clear. But, the PA was posterior and was completely committed to LV. There was a perimembranous malaligned VSD, with 25% override of d-posed anterior aorta. The mitral valve was clearly discontinuous with the posterior pulmonic valve. I reported it as TGA. However, my senior consultant changed the report to DORV based on the mitral-pulmonic discontinuity. Is it acceptable to call it a DORV when the posterior great vessel is totally committed to LV? Is the discontinuity clause a part of any Double outlet physiology or meant only for DORV? Since the number of DOLV are less, none of were very sure of this. I would like to know the opinion from the readership on this issue. If you come across such a picture, how would the report go? Please let me know.

TAPVC INTACT IAS

It was the second time in the recent past that we have come across a baby with TAPVC and intact IAS. The first one had a non-restrictive VSD and MR. The second one was latest – cardiac TAPVC with no detectable ASD or VSD and with only a restrictive PDA on echo. The surgical table found the final truth. There was indeed a small PFO, no VSD, restrictive PDA. But the surprise element was a partially unroofed coronary sinus, which went unnoticed on echo. It was very nice to know how the nature devises methods for the survival of the life. Any TAPVC with no flow across the MV is not known to survive, making the ASD near mandatory. Among the children of TAPVC with no ASD in our observation, the first one achieved a flow across the MV by MR and second one by a partially unroofed CS. It would be interesting to know the experiences of the readership. If you have come across any TAPVC with intact IAS, please let us know what other lesions led to a successful survival of the baby.

PRIMARY PROBLEM

Sometimes, certain situations can be perplexing with opinions on both poles. We had a 4-month-old with a large VSD shunting Right to Left, along with dilatation of RA and RV. There were no syndromic associations in this baby. It is very likely that the primary pulmonary resistance in this baby never dropped. It is extremely unlikely that the baby has already into Eisenmengarization. Now, in such scenarios, any further investigation may not yield useful information. How should this baby be approached? One end of spectrum is for a trial of sildenafil. On the other end lies suggestion for surgery. What way the readership vote? Is there a via-media way too? Please let me know your approach.

PEDIATRIC CARDIOLOGY PEARLS:

6. In a patient with typical auscultatory findings of an ASD and a P-wave axis of <30 degrees on the electrocardiogram, one should think immediately of a sinus venosus defect (Davia et al AHJ, 1973 page 180)

7. AV Canal defect is found commonly in children with Down syndrome. However, few specific characteristics are noted in Down syndrome:
• Situs and splenic anomalies are rare.
• They usually do not have associated LVOT obstruction, left ventricular hypoplasia, coarctation of the aorta, or additional muscular VSDs.
• Balanced AV canal defect is the commonest variant
(Marino. Cardiology in the Young, 1992, page 308)

8. Tachypneoa is a common symptom found in heart lesions with increased pulmonary blood flow (eg: ASD, VSD, PDA etc).
• In the absence of infection, the cardiovascular basis for the respiratory symptoms in such conditions probably is pulmonary edema of mild to moderate degree with elevated pulmonary venous pressure and decreased lung compliance.
• In infants with a large left-to-right shunt secondary to a VSD, dyspneoa occurs when the mean left atrial pressures reaches around 15 mm Hg.
(Donald. Progress in cardiovascular diseases, 1958, page 298)

9. In the fetus, the right ventricle ejects about two thirds of combined ventricular output, and because lung flow is only 6% to 8%, the ductus arteriosus carries 55% to 60% of combined ventricular output. (Heymann et al, Physiology review, 1975, page 65)

10. In any child with LV dysfunction, ECG is mandatory. If the ECG shows abnormal Q waves in leads I, aVL, and precordial leads V4 to V6 with or without abnormal R waves or R-wave progression in the left precordial leads, the likelihood of ALCAPA (Abnormal Left Coronary from Pulmonary Artery) is very high. All such children should undergo immediate Echocardiography in expert hands with an echo machine offering maximum resolution. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 708)

With this, we come to the end of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am yet to receive comments on improving the new segment. Please ask your general pediatric friends about the blog and get their feedback.

Regards

Kiran

2010-04-13T23:27:00.001+05:30

Welcome to NH Blog. This is Dr Kiran anchoring you to this journey. We shall continue with anecdotes that convey more meanings.

I recalled this story when my erstwhile boss was narrating her experience in one of the hospital as attendant of a patient.

Divya was a cherubic, bubbly girl of 4 years. She started going to a nearby school. She was supposed to come back from the school with another baby from the neighbourhood along with an attendant. One day, the attendant of the neighbour boy told Divya’s mother that Divya refused to come with him and she is staying back in the school.

Her mom was surprised. Although Divya was naughty girl, she never did anything similar before in the past 4 months of school. A bit tensed, her mom walked towards the school.

At a small bench near the play area, Divya was sitting alone. Her face was not its usual bright.

“What happened to you?” demanded her mom.

“Nothing, mom”, replied Divya, still with some gloom on her face.

“Did anyone say anything to you?”

“Nope!”

“Then why are you not home yet? Why did you refuse to come with that uncle?” her mother sounded angry now.

“Actually, mom” Divya started. “My friend, you know, dropped her doll and it broke”

“So?”

“I was with her.”

“Did you stay back to repair her broken doll?” ridiculed her mother in a taunting way.

“Oh! mom. I don’t know how to repair her doll. I just stayed back when she was crying”

“Which friend? What is her name? Where is she now?

“I don’t know her name. She is my friend. She left just now with her dad. I saw you coming and stayed back.”

“What did you say when she was crying?” asked her mom, getting more curious now.

“I did not say anything. I just cried with her”

Her mom was speechless with overwhelming emotions.

It is quite satisfying to have a person standing by you silently in times of agony. It is probably one place where the hospital management has to look at. We usually have indifferent staff, busy nurses, super-busy doctors and all the other people in the hospital. Management is usually engaged in the balance sheets. Hardly anyone thinks of the situation from the point of patient’s family. Should we really face the situation head-on as a patient before realising the feelings they go through? That is too expensive way of learning things. Can we be more sympathetic for situations which demand common emotions from the entire humankind? The little girl in the above anecdote is probably teaching us a superior method of living as humans.

Let us get back to the section of interesting learning scenarios once again:

TOF with TAPVC

We recently had a couple of children with abnormal pulmonary venous connections associated with tetralogy of Fallot. First baby which was 1-year-old had two pulmonary veins entering the RA and the other 2 entering LA. This had gone to OT table, unnoticed in the echo. Second one was a much higher shocker! This 4-year-old had very poor echo windows and cath study was suggested to evaluate for additional VSDs. In the cath, the RA catheter entered RUPV. Hand injection of contrast showed the dye entering RA. The surgeon was informed about the association of PAPVC. However, on the table, the surprise was revealed. The baby had TAPVC! Was the hand injection of dye in cath not enough to opacify common confluence? We went back to the echo recordings, but the windows were so poor that we could not get any information on pulmonary venous return, especially in this baby with very small Qp. I am seriously considering a prospective study on the differences found on the OT table against the final diagnosis provided by the cardiology team. How are the experiences of other centres? Please let us know your experiences.

CHANGING PATH(OE)S!

We came across a 3-year-old operated for Tetralogy 2 years back. The boy was followed up in his native and came to us for the first time after the surgery. He was otherwise doing well, with no symptoms. On echo, we could not find LPA either in 2D or colour in any of the conventional views. The flow across RPA appeared normal. However, on clinical examination, his left lung had normal breath sounds, no added sounds. We wondered why the LPA was not visible. Small clue on chest radiograph was that the Left dome of diaphragm was high up with normal vascular pattern on both lung fields. It was probably paralysed during the surgical procedure and must be partially recovering now. It was likely that the orientation of lung has changed in such a way that the LPA must be traversing in a very odd angle and limited the visibility of LPA. This was only a hypothesis and we could not find any better explanation. We did not get any further investigations, as the clinical picture was very satisfactory and virtually no clues of abnormality in physical examination. Any experiences with such a pattern? If anyone has any better explanations, I would like to hear. How do you think such kids should be investigated? Please let me know your opinions on it.

NO COMPACT OPINION

Many times, we come across number of children with ventricles looking similar to non-compaction. What are the actual criteria for this diagnosis? Is a simple visual impression enough to say this? Are there any clear cut guidelines to say so? The text books describe it as spongy appearance of ventricular surface with thin epicardial layer and a thickened endocardial layer with prominent trabeculations and deep recesses. How to objectively evaluate this entity, which has only visual description? How many times do we really see ventricular dysfunction associated with this picture? How are the experiences with other centres? Please let me know your take on it.

DOUBLE DYSPLASIA

It was a child which disturbed our team a lot. This 3-month-old boy had truncus arteriosus. However, the truncal valve was quadricuspid and dysplastic. There was no other reason why this baby should not have had further procedures. However, is it worthwhile to convert this leaking dysplastic valve (which is not even repairable) into the future aortic valve? Is there any other option than rejecting the scenario as “untreatable”? Has anyone come across a similar phenomenon earlier? Please let me know your views on this.

REGURGITATIONS IN OPERABILITY

On an interesting note, we had three children with AP Window on a single day in our OPD. It was more than a coincidence. One among them was a 9-month-old with type 1 APW. This child also had mitral and tricuspid valve prolapses with severe regurgitations along with moderate aortic regurgitation. The net result was dilatation of all the cardiac chambers with reversal of flow in the aortic arch. The pulmonary venous return on the echo could not be assessed satisfactorily. Now the question was: How would one assess the operability on the echo for such children? The child saturated 93% in room air. Is that enough to claim operability? Is the cath study mandatory? Let me know how you would handle this issue.

Some of my friends who are pediatricians with lots of academic interest had an objection about the blog. They claim that it is too much of pediatric cardiology alone. They wanted something that would interest the general pediatric person with interest in Pediatric cardiology. Also, the post-graduate students in pediatrics wanted something that would interest them. With these in mind, I have decided to start a new section from now on. This section would be called “Pediatric cardiology pearls”. I would give 5 interesting and practical points in pediatric cardiology from a known work, along with the source. It should be good for the practitioner and students for management and quoting in the scientific meetings as well. Here goes the first instalment:

PEDIATRIC CARDIOLOGY PEARLS:
1. In the ECG of ASD, one can often see a notch near the apex of R wave in leads 2, 3 and avF which resembles the croche needle. This is called the crochetage sign. This has high specificity for the diagnosis of ASD if found along with the rsR’ pattern in the ECG. Contrary to the popular belief, the crochetage sign does not have a steady correlation with the shunt severity and is often found with PFO also. (Heller et al in JACC 1996, page 877)

2. An Eisenmenger VSD (also called Eisenmenger malalignment) is a perimembranous VSD with an anteriror deviation of infundibular septum. This has nothing to do with Eisenmenger syndrome or Eisenmenger complex (shunt reversal due to high pulmonary vascular resistance). (Fukuda et al in Cardiology in the Young 2000, page 343)

3. PDA is classified into 5 types: wide aortic end, wide pulmonary end, tubular, multiple constrictions and bizarre. (Krichenko et al AJC, 1989, page 879)

4. In the AV canal defect, distance from the cardiac crux to the left ventricular apex is foreshortened, and the distance from the apex to the aortic valve is increased. As a result, the LVOT is longer and narrower than normal and produces the “gooseneck” deformity. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 646)

5. A combination of persistent left superior vena cava terminating in left atrium, atrial septal defect, and absence of roof of coronary sinus is called Raghib complex. It is associated with cyanosis – one of the few causes for cyanosis in ASD without Eisenmengarization (Raghib et al Circulation 1965, page 912)

With this, we come to the conclusion of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. Thanks for all the new followers. Please let me know how the new segment can be improved with the defined objectives that I have mentioned.

Regards

Kiran

2010-03-11T23:27:00.000+05:30

Welcome back to NH blog. This is Dr Kiran. We are in the process of finding something pertinent to our medical lives hidden in some of the known anecdotes.

The following anecdote was quoted by one of my friends. He had read it in a magazine few years back. He found the end very touching and narrated to me.

The story goes like this:

Mr and Mrs Shaw were worried. Unlike other members of their family, their youngest son Rahul was not an academic achiever, despite reaching his fifth standard.
The parents gave him everything a child could desire. Their only motive was to make him an academic success. They wanted their son to top the class, as did their other children. They were telling Rahul the virtues of good academic career. They would quote how his elder siblings are respected in the family for their academic excellence and so on. Not that Rahul was stupid. He was naughty, mischievous, playful, spoke a lot of nonsense, played well, but could not study and get good grades. His teachers were fed up with him. They would often call the parents to school to report him. They advised the parents to seek the help of a counsellor to rule out attention deficit hyperactivity. Many teachers could not just bear his presence in their class. All these were reflected in his report card as low grades. The school authorities threatened the parents of a transfer certificate if they did not get any better of him.
And, suddenly Rahul became better. His grades started improving. His attention span increased. There were literally no complaints from the school for more than a fortnight. It all sounded and felt magical for the parents. They had not done anything from their end, but still, something seemed to work.
This time when the parents were called to the school, they expected another taunt, but were taken by a surprise. The teacher had called them to congratulate on their effort. Rahul had got good grades in the unit test. The parents could not digest this. They started feeling guilty for the appreciation bestowed on them for no effort of theirs.
But the event deserved celebration. Both parents took Rahul for an ice-cream. In the parlour, Mr Shaw asked his son: “How are you doing all this?” He was worried if his son had started copying from his neighbour.
“I did not see anyone’s paper. It is all my own effort” said the lad.
“But how?” demanded Mrs Shaw this time.
“I have got a new friend. She works in my school. I talk to her all the time. I feel great with it”
“In the school?” thought Mr Shaw. “If the effort is done by the school to improve him, why are they complimenting us?”
“Something’s not fitting” he told his wife. “Can you show me your new friend?” he asked his son.
“Of course. She would still be at school. We can go now and meet her” said Rahul with a happy note in the voice.
They turned their car towards the school. Rahul led them towards the library. They found a lady in her twenties, arranging the books in the racks.
“What is her name?” asked Mr Shaw.
“I don’t know” was the prompt response from the son!
They waited till the lady was done with her work and returned to the counter. She saw Rahul and looked happy.
“These are my parents” introduced Rahul.
The lady bowed to them gracefully and smiled.
“Thanks for helping my son. We don’t know how to thank you enough”, said Mrs Shaw gently.
The lady did not say anything. She showed few gestures with her hands, which neither of the Shaws understood. All that they could make out was that she was using sign language used by verbally handicapped.
“Oh my God! She cannot speak?” said a shocked Mrs Shaw.
“Not just that. She can’t hear, read or write either. She is employed by the school to keep the library clean” said the Library in-charge who was passing by.
The parents looked perplexed. Mr Shaw came out of the shock and demanded with his son: “What sort of help have you got from her that improved you so much? She can’t teach you any academics; she doesn’t know how to write. Forget other things – she can’t even hear you.”
Smile could not leave Rahul’s face. “She does far more than hearing, Dad. She LISTENS!!”

We often miss this point. Most of the times, we medical professionals hear or preach. We do not listen. I have a nice recall from my medical school times. One doctor who was considered just mediocre for his medical knowledge was very popular with patients. Similarly, another doctor we used to adore for the clinical acumen and great subject knowledge was a total flop with patients. When this was discussed amongst friends, we would flatly declare that the patients are foolish; they are so ignorant and dumb that they can’t even recognise a good doctor from a bad one. We felt that we knew the definition of “good” far better than the patient.
On the hind sight, now I feel that the patients were not outright foolish. They sought what they wanted and got it. The doctor who was mediocre in our view was good for them, because he LISTENED. Our “great man” either heard or preached. We often witness how emotional catharsis helps the patients in psychiatry. But, we fail to extrapolate the same for ourselves. Many a times, the patients need a soothing soul to pour out their inner self. A good listener. But how many of us can afford to do so in our busy practise? When time becomes money for the professional, where is the scope to be a good listener? But, there should be a way out. There are many doctors who have successfully balanced both very nicely during their entire career. If you ARE one or know one, please communicate with our readership. Let the world “LISTEN” to you this time.

With this, let’s get back to our regular feature: Challenges in Pediatric Cardiology!

VIAGRA CONTROL

It has been few years that we are using sildenafil for the pulmonary hypertension and high PVRI. Indiscriminate use is one of the biggest challenges bothering the congenital heart care. We have seen doctors equating PAH with sildenafil. Despite all the efforts of journals and experts trying to create awareness on judicious use of sildenafil, the misuse is rampant. Is there is any role of Bosentan? How are comparative analogies between Bosentan and Sildenafil? Please let us know if any studies on this comparison are published.

LIMITATIONS OF VALVE

We had a 32-year-old with small subpulmonic VSD, bicuspid aortic valve and moderate AR. The issue was with the valve. No doubt that the VSD needs closure as per guidelines. But, is the valve repairable? If yes, then the effort is worth it. If not, then the patient needs valve replacement. Is the valve replacement worth in a 32-year-old, especially when the hemodynamic effect of VSD is small? What happens if the lesion is left untouched now? Is it possible to close VSD alone and not attempt the valve repair at all? All these questions bothering us, the patient data was placed in front of the surgeons. The surgical team decided to go ahead with the VSD closure and assured that the valve can be repaired. Their analogy was simple. If the VSD is closed with the AR left alone, the natural history of bicuspid aortic valve would be far superior to that with the VSD pulling the aortic valve cusp. If VSD is not tackled now, the patient may deteriorate faster. What is the experience with other centres? How would you tackle this scenario? Please let me know.

CONVENTIONAL CONFUSION

Sometimes, we wonder how fast we forget! Once we reach a level of practical comfort, the theory that was read becomes easily effaced. Same happened during one of our seminars recently. All of us remembered the classification of Tricuspid atresia as I, II, III based on great artery relations and A, B, C based on the pulmonary valve and pressures. Although I am quite fond of history, I had forgotten that the mentioned classification goes with the eponym of Kuhne. Our fellow, Dr Shweta, was presenting the seminar. She told us that the class IA was intact IVS AND pulmonary atresia, class IB was restrictive VSD AND pulmonary valvar stensosis. I wondered if the term AND is correct. The feeling was that AND should be replaced by OR/AND. However, she showed the reference from the text book (Moss and Adams). I was unable to get the original article by Kuhne et al. If anyone has any recall or has the original article, I request you to pass it on for the benefit of others.

LOCATION, LOCATION, LOCATION

I am yet to find a person who has completely understood the VSDs! This simple looking lesion can pose so many surprises, that the moment you get comfortable with it, a new problem, hitherto not experienced, pops up. In one of our discussions, our senior surgeon opined that the outlet VSDs have development of early PVRI compared to VSDs in other locations. The opinion was seconded by others. I could not recall any statement of this sort in the texts. I presumed the probable reason to be the proximity with pulmonic valve. It is likely that the hemophysiology of outlet VSDs simulate AP Windows to a certain extent. Is my presumption correct? If anyone can come with proper explanation with proof, you will be rewarded by a special mention in the blog; your name would be written in CAPITAL LETTERS!!

BLUE OR NOT

We were coursing through some of the interesting cases seen off late. We do have a series of single pulmonary arteries unpublished yet. One of such cases was a hemitruncus with intact septae. The question was: would this patient ever by cyanosed? If yes, why and how? I was emphatic that such patients would never have a cardiac cyanosis. The pulmonary artery exposed to the high pressure would eventually develop high PVRI and restricts the blood flow. The entire right heart output is going to single lung and it may eventually develop high PVRI. Till then, the patient should be pink. After bilateral high PVRI, the patient may have signs of low cardiac output and failure rather than cyanosis, unless the PFO opens up or pulmonary AV fistulae develop. Hence, clinically, the loudness of S2 would determine the status of the patient. Soft S2 shows patient has at least one good lung and loud P2 shows the development of high PVRI in the hitherto good lung. However, there were some arguments against my logic. What is your opinion? We may not have seen such a scenario, but may logically conclude the outcome. Let me know your takes on this issue.

This scenario marks the end of another post. Let me get the suggestions and answers to all these queries. Put them in the comments box or mail them to drkiranvs@gmail.com Let’s see how best this platform can be utilised for the purpose of mutual learning.

Regards

Kiran

2010-03-09T23:16:00.002+05:30

Welcome to NH blog. This is Dr Kiran.

From last post, we decided to put some anecdotes which carry a greater meaning than what meets the eye. In the attempt, I have picked up this small story one of my friends told me once. She also had no recall where she heard the story. It may very well be getting transmitted verbally from many. If this story is in print anywhere, I would be indebted to know the same from the readership.

The story goes something like this:

A board hung on a pet store caught the attention of this young boy of nine.
PUPPIES FOR SALE
Boy slowly went into the shop and smiled at the plump owner.
“How much are the puppies?”
“It would be Rs 300/- each. We have left with only seven more. Have cash or wanna come back with dad?”
The boy meddled with his pockets, brought out some change and counted.
“I have Rs 45/- only now. Can I see the puppies?”
The store owner was a kind man. “Come on in!” he said.
As the boy got in, he found seven cute creamish puppies running hither and thither. A smile laced his face. To his surprise, he found the eighth puppy limping along.
“I heard seven. But they are eight out there!”
The shop keeper looked down the place and said:
“Oh, that’s a defective one. We showed him to the doctor. This puppy has some problem in legs by birth. I have not included him in the list”
“Will he always limp like that?”
“Yes, my boy. Thats why I did not tell you about him”
“I want to buy him!” the boy said.
“No point, child. I don’t even have him in the list. I don’t want to sound cheating a little boy. If you still think you want him, I will give him to you for free.”
The boy’s face grew red. He sounded angry. “Please don’t use the word ‘free’. I don’t want him free. I want to buy him. He is worth the same as all his other siblings. You please keep this Rs 45 as advance and book him for me. I will send my father to pay the rest.”
The shop owner was perplexed. He also had the admiration for this small boy at the same time. He tried to convince the boy.
“I think you should consult your parents before making this decision. You see, this puppy is never going to be a normal, playful one. You will never be able to take him out for fun. There is no chance of you jumping and playing with him.
The boy smiled, “You are wrong”. He slowly pulled his pant up. It showed steel braces around his deformed leg. “It is not about this puppy. Even I can’t run around and jump either. We make a pair because we can understand each other!”
The shop-owner was speechless.

It takes lot of empathy to understand the feelings of patients’ companions. It becomes glaring when the tables turn. I remember our erstwhile boss writing to all of us when her father was in the ICU of a prestigious hospital at Hyderabad. She came back after the demise of her dad and was so full of new ideas about patient care and communication. She could feel for all the parents waiting in the lobby just to hear something about their patient by one of the personnel from inside. She emphasized the need for more frequent, easy and meaningful communications with patient party. She urged the importance of supporting the family at the times of distress and impending death. She conceived the idea of keeping the patient happy till the last moment by creating an atmosphere of solace for everyone. She suggested that we should allow the parents to stay bedside during the last moments of the child and the mutual satisfaction that can be provided when the parents can hold the baby’s hands in those moments. She mooted the proposal of making the end as beautiful as possible for the tender hearts.

Not that our brilliant boss could not have thought of all these issues prior. She probably had in bits and pieces. However, everything got crystallised into a solid structure when she went through the experience with tables turned around. She could empathize with her patients better after her experience with her father’s demise. One example of how a committed person would see the changes around with a positive outlook.

Let me know your views on it. Also, if you have read or heard of small anecdotes, please send them to me. Your contribution would be promptly acknowledged!

With this, let us get back to our regular feature: Challenging scenarios.

IS THIS TRUNCUS POSSIBLE?

I remember sometime back when a cardiologist from a small volume centre presented Truncus arteriosus with intact interventricular septum. We were smiling at the possible embryological nonsense. The shock was on us when we saw a baby with same diagnosis. Our surgical team questioned the well being of cardiology team when we mentioned this to them. We showed them the echo images to stun them. Our enthusiastic fellow, Dr Karunakar had already gone a step ahead and pulled out a couple of articles from his bag on the previous case reports of Truncus with intact IVS! The question was of the management. This 2-month-old was not very well at presentation. The surgeon went through the literature and had a couple of plans for him based on the anatomy on the table. Sadly, the baby did not make it to surgery and expired in the ICU. This is the first time we had come across any Truncus with intact IVS (we have operated 115 cases of truncus so far at NH). Please let me know if you have seen a truncus with intact IVS and how it was managed.

UNANSWERED McGOON

This has been a perpetual question that might have haunted generations. Probably, I have posted it earlier also. When one of the pulmonary arteries is far smaller than the other and the McGoon’s ratio becomes acceptable, what is the approach to be taken? We often see the surgical and cardiology teams split on the issue. What are the opinions from other centres? Please let me know your expertise in it.

WHOLE OR HOLE?

We had an eight year old with DORV, d-malposed great arteries, VSD, pulmonary atresia and intact IAS. This child saturated 78%. Although there were 2 good sized ventricles, the VSD was not routable. The question was whether to create a BTT shunt or to go for a BD Glenn shunt. The house was spilt on the decision. One half felt that the BTT shunt might be better, as BD Glenn has long term issues. The other half felt argued that the volume load on the ventricle by a BTT shunt would be detrimental on a long run and BD Glenn would serve more than one purpose. It was decided to measure the PA pressures on the table and go with BD Glenn if OK; otherwise to settle for BTT shunt. The other interesting question was – whether an ASD would be required in this case? Since the aorta was more committed to RV and VSD was just reasonable subpulmonic type, should this baby have an ASD to compliment mixing? This was a tough question to answer, as the surgeries proposed were off-pump and to create an ASD, one need to go on-pump. The surgical team could not recall any similar situation from their kitty of experience. Neither was the cardiology team sure of the answer. If anyone had such experience, please enlighten us.

BEHAVIOUR: BEFORE AND AFTER

We had a couple of children with VSD, PS, small RV and had gone through BD Glenn shunt few years back. Now, they returned with progressive restriction of VSD and symptoms. RV was suprasystemic on cath study. The RV EDP had gone up beyond the permissible level for Fontan completion. Although the Glenn shunt was still partially functioning, it was probably a matter of time. How should one go about such situations? Creation of a larger ASD may serve them for few more years and not a long term solution. Is there anything like enlarging or creating a VSD? The surgical team quoted “Prohibitive risk”. Is it the final word? Is there anything that can better such situations? Please tell us about your experiences.

ORTHOTOPY Vs HETEROTOPY

An interesting observation was quoted by one of our senior cardiologists, Dr PV Suresh. Amidst his ever-flowing OPD patients, he had observed the natural history of pulmonary homografts in Rastelli Vs Ross procedures. His observations showed that the Ross homografts behave far superior to the Rastelli homografts. He was seeking the possible explanations for this. The surgical team was represented by Dr Shekar Rao, who has exhaustive experience in both the procedures. Apart from the individual magnitude of both procedures and patient selection, Dr Shekar Rao quoted the advantages of orthotopic positioning of Ross homograft and possible disadvantages of heterotopic positioning of Rastelli homgrafts. The discussion was interesting for senior group and eye-opening for the freshers. We ended up understanding the hemodynamic variations of a 3-D angulation of cardiac structures. It is always emphasized that we cardiologist should have a mental 3-D reconstruction of anatomy with the 2-D data what we get from conventional imaging. This time, we could gauge the 3-D physiology also! We are indebted to both our senior consultants for the new light. Not to boast of ourselves, that’s probably what makes NH a sought after place for students!!

END OF AN ERA

After going through a series of emotional tides, the NH team is settling now. Dr Sunita Maheshwari, our beloved “Boss” decided to quit her post from NH to make space for her dreams. The decision was greeted with surprise, anguish, shock, anger, sorrow and mix of emotions from other team members. The Fellows were not very happy with the development, although it does not affect them on a long term. Such was the gravity created within. On the day of commemoration of a decade of Pediatric cardiology in NH, we also witnessed the handing over of charge to Dr PV Suresh. Although Dr Sunita has promised to come once a week for teaching, we know it is not long term happening. Personally, I feel that Dr Sunita’s achievement lies in the fact that she has prepared the department for her absence. It is no doubt the sign of a leader with a positive vision. Now, it is left for the team to honour her by achieving greater heights despite her absence. That’s one way I think we can thank her. We all wish for an uninterrupted flow of her creative energies and scaling of greater heights she is ever capable of. Adieu.

That brings us to the end of another post. I am yet to receive any comments for the new effort. Please let me know if it is worth continuing. Come out with any better formats for presentation. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership. I hope to get more frequent henceforth.

Regards

Kiran

2010-02-21T22:55:00.001+05:30

This is Dr.Kiran welcoming the readers to NH blog. The blog aims to put something "old and fresh" of Pediatric Cardiology everytime and invites the readership to actively participate in the dialogue process.

Let me render my apologies to begin with. It has been more than one month that I have posted anything. I was a bit busy with an article for publication, under a strict ultimatum! Howsoever small the readership might be, I owe this explanation to them. Hence this.

So far, we have seen something about the historical developments, Nobel prize winners and historical developments of drugs: all in relation to Pediatric Cardiology.

I had run out of the ideas and asked the readers on some suggestions on the topics of possible interest. As usual, I have got none! So, I have tried to do something which was suggested sometime back by one of the very good friends and a fabulous team-member, Dr Vishal Changela. He strongly felt that some personal medical anecdotes about our professional relationship with patients should be put up for others to read. In the absence of any other better things to write, I am trying this from now on.

Long back, I had heard a short story written by one of the greatest short-story writers of Kannada, the legendary Dr Masti Venkatesha Iyengar. It is a simple village story. One evening in one of the villages neighbouring a forest, a cowherd realises that a buffalo and its calf are missing. Since it is already dark, they fear entering the forest in search of the missing duo. He keeps his fingers crossed and waits till the dawn. A search party with the armamentarium enters the forest carefully. After a couple of hours of search in the deep forest, they hear the hunger cries of a calf. They start towards the sound and find the same calf of the buffalo safe and secure. In a short distance from this, they find the wounded buffalo in a pool of blood, breathing heavily. To their shock, they find the paw marks of tiger moving away from the spot with blood splattered all along its course, disappearing into the heart of the forest. They immediately realised the entire sequence of events. The calf must have wandered into the forest unknowingly in the late evening and its mother must have followed it to safeguard the kid. A tiger must have attacked the calf and the docile buffalo had fought the mighty tiger tooth and nail throughout the night to save its baby. It finally succeeded at the cost of its own life, driving the deadly beast wounded and away. Just imagining a tame buffalo fighting a ferocious tiger in the night to protect its calf keeping its own life on stake was nerve wrecking and emotions filled the souls of those poor peasants. The villagers carried the wounded buffalo and the calf back to the village. The buffalo eventually died and the villagers buried it with full honours that befitted a brave person and had a small monument built in the memory of its love and courage. This true story stands as a phenomenal symbol of selfless sacrifice of a mother for its child.

The story brings about various kinds of emotions in the reader. Few imagine the scenario and feel for the motherhood. Few imagine themselves in the scenario and try emoting. Few extrapolate the real life situations and think of cross references. What comes out an anecdote is your experience and empathy inside. We all feel for the sacrifice of the buffalo largely because of the courage this helpless, docile and mute animal showed at the time of danger and crisis to its calf. It is probably the basic nature of this animal that stands contrast to the courage it displayed, which fills the mind.

We do see situations in the hospital similar to the one the anecdotal buffalo underwent. We see parents who would sacrifice what all they can to get their children treated. We see them selling the last piece of jewellery, spreading their hand in front of the last available donor, saving money by choosing the least expensive accommodation for themselves or taking just one lunch and so on when the child is admitted in the hospital. All the gruesome effort happens despite accepting the risk and keeping the outcome sceptical. The sacrifice appears to be hanging only to a 4-letter word: HOPE. I am often reminded of the anecdotal buffalo whenever I come across such situations. The scenario of the otherwise helpless buffalo is no different from helpless parents here. The buffalo fought with mighty, cruel tiger and the parents here fight with mightier, more cruel and more than that - an unseen destiny. I feel the sacrifices on both occasions are no less than each other. The first scenario got noticed. But the latter goes unnoticed all the time.

These are the kind of anecdotes that we would like to present. It need not always be tragical. Some triumphs can also be interspersed. At finale, what re-shines is the humanity. The Mahabharata says, “there needs to be only one religion and one principle in the world: compassion to the fellow life”. It is said that the humanity re-lives every time it triumphs. If you have any such anecdotes, please send them to me via email to drkiranvs@gmail.com I shall acknowledge your contribution and give full credit to you for your story!!

I shall get back to our regular feature: Interesting and perplexing case scenarios

TO OR NOT TO TIGHTEN

When the pre-Glenn shunt cath procedure is done, we often see PA pressures being borderline high, but all other parameters would favour Glenn shunt. In such scenarios, few suggest tightening of MPA and going ahead with Glenn. It may get the circuit OK for Glenn, but may reduce the SO2. Is this acceptable? Isn’t good SO2 one of the objectives of Glenn? By tightening the MPA, do we always achieve the Glenn suitability? If anyone found answers for these, please let me know.

BALLOONING IN REGURGITATION

In cases of severe Aortic stenosis with moderate aortic regurgitation, can we consider ballooning? We had a 13-year-old with this scenario. His aortic annulus was very small and looked mildly dysplastic. Surgical team felt that placing a good sized aortic prosthesis was not possible. Doing a Konno was considered very risky. Annuli were not suitable for Ross. In such cases, can we contemplate controlled balloon dilatation, accepting the eventual AR? Our senior consultant was of the opinion that free AR would be very dangerous in such children and balloon should not be attempted. Is the natural history in this subset any different from any kind of intervention? What would you do in such cases? Please put your comments on it.

Qp OR Qep?..

Numbers are funny. We have seen hair-splitting episodes on the basis of numbers generated after gruelling procedures! Whether operability or fitness for univentricular procedures, numbers do play a vital role in this field. During the combined cath meeting, the house is split many a times on this factor alone. We often find children cathed for operability. When the shunt is bidirectional, as per the standard references, we use the effective shunt – Qep. The Qep, many times, is significantly different from the Qp. In all such cases, should we use Qep or Qp as the denominator for calculating the PVRI? This alone can make the difference for the patient. We had a 7-year-old with VSD, PDA and PAH, whose operability was doubtful on the clinical grounds. Basic investigations could not be deciding. Cath showed reasonable Qp/Qs, but the PVRI was marginally high. Our team felt that the child should be operated, as this might be his last chance. Surgical team quoted the higher number PVRI and mooted inoperability. We were no sure how much to believe the numbers when the denominator was not fully certain. Is there a correction factor to the formulae when Qep is involved? Let me know your experience and literature back up if any.

PLACING THE BAND

An interesting analogy was discussed by the surgical team in one of the meetings. It is probably not textbook written and might be more experience oriented. While witnessing the angio images of a child who had undergone a PA banding as an infant in a hospital abroad, one of the senior surgeons commented that the said PA band was meant for a future single pump physiology. We were surprised how he could be so certain, as he was seeing the details of the patient for the first time! Then the surgeon pointed out that the PA band was placed close to the pulmonary valve, which is done to prevent distortion to the branch PAs. Similarly, he said, the PA bands are placed close to the bifurcation if a future 2-pump repair is contemplated. We were not certain if that was the rule, as we were considering both options in the present patient. Other members of the surgical team agreed with this. However, I could not recall reading about this anywhere, nor my cardiology colleagues. If anyone has read something about this, please enlighten us.

SINGLE VENTRICLE: WOULD LV/RV BE A FACTOR?

This was an interesting one. We had a 5-year-old with tricuspid atresia IIIC, who had undergone a PA band as an infant and saturating 88% at present. We had suggested single pump repair for the child. She fulfilled the criteria for single pump repair. However, the surgical team felt that the child can be followed up, as the saturations are OK. We argued that the long-term ventricular load should be the main consideration for present surgery. At this point, one of the senior surgeons commented that he would have taken it if it were to be a mitral atresia instead of tricuspid! He was of the opinion that the volume load on the ventricle would be a factor only when the pumping single chamber is RV. As this patient would have LV as the pumping single chamber, volume load should not be a consideration for present intervention. In any of the criteria of single ventricle, this factor does not find a place. We were using this logic for long term prognosis of single ventricle repair, but what our surgeon told appeared new. It may sound logical, but is there any consensus among the surgeons on this? How would you tackle this situation and argument? Please let me know your take on this.

TWO IN ONE?!

We had an infant with dTGA, ASD, small VSD and severe peripheral PS. The branch PAs appeared to have a tortuous course. The echo study alone was not enough to understand the branch PA anatomy. It was not very clear if they had multiple constrictions. From our side, we were not in consensus on the plan. We decided to place the scenario in the meeting. We showed the echo images and asked the surgeons to comment on it. Although it was decided for a close follow up at present, one of the surgeons felt that the LeCompte manoeuvre done during arterial switch can straighten the branch PAs and they may not require any additional intervention! This was hitherto unheard of! Again, sounded logical, but is it the fact? One can understand that the logic would work if the branch PAs have just folded upon themselves. Would the same logic apply if there are anatomical constrictions in the branch PAs? If anyone had any similar experiences earlier, please let us know the outcome and the way you went ahead with the plan.

That brings us to the end of the present post. I have tried something different; totally different from my areas of strength! If there are any differences of opinion, please let me know. If you think any better formats for presentation, please suggest. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership

Regards

Kiran

2010-01-14T22:35:00.003+05:30

Readers are welcome to the NH Blog. We are in the process of learning something about the developmental history of cardiac drugs. This time, we shall see the history of Aspirin in regard to the heart.

Before we start, I have some news which made us proud. Our boss, Dr Sunita Maheshwari was featured on the Cover of the prestigious India Today Woman in the January 2010 issue. (Incidentally, the accompanying regular issue of India Today had Aamir Khan on the Cover!) It was a matter of pride for the entire institute.

The second news was the glory we basked in the Madras Medical Mission. The annual teaching programme of MMM for the DM equivalents was held a week back. Our senior fellow, Dr Vishal Changela got the award for best case presentation. Our fellows literally dominated the session and no answer got missed from them. It stands as the solid testimony for our training and the quality of our fellows.
With this, lets go back to our regular content of blog.

One of the finest accounts of the history of a drug was written by a scientist who was directly involved in the development of the drug. Written in German, its English translation is as fascinating and soul-touching. The author of this historical account is Arthur Eichengrun, whose original article was published in 1949 in a German journal called Pharmazie. It is a “must read” for any enthusiast of medical history and (in my personal opinion) for everyone. The drug is the ubiquitous ASPIRIN. What we shall see is the development of cardiac utility of aspirin, skipping the anti-inflammatory history. For those who would be interested in the entire legacy of Aspirin history, I would strongly recommend a fantastic article by Walter Sneader in BMJ in the year 2000.

Lawrence Craven was a busy surgeon in California. His busy schedule would allow him minimal time to interact with patients and any question of personal touch was usually ruled out. On one of his busy day, he performed a tonsillectomy for a boy. Unable to get in touch with the surgeon after the surgery, the parents of the boy offered him a chewable aspirin for analgesia. The young patient started bleeding, worrying his surgeon for possibility of re-exploration. However, good sense prevailed and the surgeon decided to wait. Under some stern questioning, the parents spilled the beans and the surgeon went into a thinking mode! He made enquiries with other patients asking just 2 questions: Have you taken aspirin as post-surgical analgesia? If yes, did you experience any bleed? To his surprise, the OTC was consumed by many without his knowledge and universally had experienced visible bleed! The scientist in the surgeon made an appearance and he decided to investigate this further. He learnt that one Gibson had tried the use of Aspirin in a small number of patients with vascular diseases with equivocal response. Craven decided to make it big, but ended up being crude.

He tried around 8000 patients who consumed aspirin and came out with a claim that would surprise a seasoned physician! He claimed that none of these 8000 suffered a heart attack!! He could not publish his studies in any major journal as the trail was not a controlled study. The anticlimax of the study was reached when Craven himself died of a heart attack even after taking aspirin. The sceptics would have ended the saga of aspirin with this irony. However, good sense prevailed again and Harvey Weiss in New York could re-establish the role of Aspirin in 1967. He categorically demonstrated the acquired impairment of platelet aggregation in the subjects consuming aspirin by demonstrating prolonged bleeding time. He was the first to suggest the anti-thrombotic nature of aspirin and its use in clinical medicine. He urged the scientists to take up a larger trail in this regard. When it happened, there was no looking back. Aspirin was proved to be an effective anti-thrombotic agent. However, it took many years to come to the right dose that was minimal enough not to cause side-effects, but sufficient enough to provide the desired anti-thrombotic effect. As of today, aspirin stands as one of the most precious drugs in cardiac pharmacology.

With this, I am concluding the “Developmental history of Cardiac drugs”. I have not received any suggestions from any of the readers (if such a population exists!!) on what aspects of history to write next. May be, for next few sessions, I shall write only interesting cases and take up the historical aspects after some thinking.

On a personal note, the year 2010 is seeing lesser number of OPDs in Pediatric side. Possibly the festive season in Tamilnadu might be the reason. We should have taken this opportunity to relax a bit, but some of the events happening never let us!

VENOUS PVRI
I have posted this query in the past also, but it continues to haunt us on a regular basis. Hence, I am posting it again. This time, the patients are different, but the problem remains the same. We had one 16-year-old boy with Supramitral membrane with severe obstruction, Severe LVOT obstruction, large PDA and coarctation. The combination fits into Shone’s complex. The CoA was juxtaductal and the PDA was flowing right to left. The boy was desaturated in the lower limb with a significant drop in the LL saturation. His calculated baseline PVRI was 22 Wood units, which dropped to 9 Wood units with oxygen. Now the question remains the same. Is he operable? Isn’t the PVRI secondary to pulmonary venous hypertension reversible after correcting the cause? How much of PVRI is due to PDA and how much due to left heart obstruction? When put to vote, only a handful of people felt it was operable. The young guns of surgical team felt that the patient deserved to be operated as it might be his last chance for repair. However, the senior rung was not so much for it. They felt that the CoA was not much significant with PDA being the dominant lesion and it clearly shows inoperability. The decision was a bitter pill to swallow, but none of us have a concrete evidence for our stand. Such cases can be argued on either side. I questioned the need for cath study in such cases, as the pressure data and interpretations are dubious (may not be decision making in black and white) and the anatomical data might be much superior with a CT and a 3D reconstruction. However, the opinion favoured the cath study over CT. When I had to counsel the parents after the cath meet, I was choking for words. I personally felt that the boy should be given an opportunity to undergo surgery, accepting the high risk. However, the uneducated parents may not understand what we mean. I directed them to the surgical team. What bothers more is the fact that the next patient with similar problem would restart the enigma and we are no wiser by the experience of the present patient. Can anybody throw some light on this? I badly need some advice on management issues of such class of patients.

BALLOONING FOR SURGERY
We came across an infant who had undergone artereial switch as a neonate. He is otherwise asymptomatic, except a murmur in the precordium. Echo showed bilateral branch PA stenosis. Cath showed a significant gradient in branch PAs. Is there a role of balloon dilatation in such cases? How far is it successful? The age may not permit us to stent. Is isolated balloon dilatation recommended? Should we go ahead with surgery directly or give a trail of ballooning? Put in your ideas.

BAND ENIGMA
This question has come up in the past, but is another case for lack of consensus. We have a 6-month-old boywith single ventricle physiology (DILV) with mild PS. His mean PA pressure in cath was 36mmHg. Now, should we be doing a PA band? How far can we offer a BDG for him in future? Is he better off as he is now? Our chief was in favour of a PA band so as to keep the future options open, but the surgical team vehemently argued against it. I thought of putting the issue with both possibilities to clear the concepts for myself! I questioned the surgical team if we can anticipate any progress in PS as a part of natural history in this subset. It was interesting to know that such incidents have happened when the level of obstruction was at restricting VSD to produce a PS in Holmes heart. My next question was on the naturally history of a scenario with present PA band, but not suitable for future BDG. Would the eventual pressure overload on single ventricle be worse than the otherwise natural history? The answer had 2 interesting facets to it. Not only such a scenario increases the pressure overload on the heart, it also increases the cyanosis, because the afterload of pulmonary circulation would be greater than that of systemic. The band enigma was another unresolved issue which made us not much wiser at the end of it.

NORMALLY TRANSPOSED
Is it possible to have a TGA with normally related great arteries? We did see newborn with this picture. It had a clear VA discordance but the aorta came out of RV posteriorly, followed by the PA from LV, without the great arteries crossing each other. On the short axis, aorta was to the posterior and right with the PA anterior and left. Now, as per the segmental anatomy, they are {S,D,S}. Do they fit into NRGA? Is the component of crossing essential for defining normal relationship of great arteries? Can the term “Transposition” be applied if the vessels are normally related? Should we call it an isolated AV discordance or TGA? If anybody has seen such a picture before and had any thoughts gone into it, please enlighten us.

TRICUSPID SHONES
Does the definition of Shone’s complex involve the mitral valve, aortic valve and arch or does it involve left sided structures? We came across a case of corrected Transposition with sequential left heart obstruction. There was a tight supratricuspid membrane with subaortic membrane and a coarctation. Technically speaking, it does not fit the definition of Shone’s complex. This is the first time I have come across a cTGA with sequential left sided obstructive lesions. Our surgical team had a tough time operating this child. Tell me your experience if you have come across this combination anytime.

Send your comments. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Write about the issues you found perplexing and the way you have found the answer for them.

Regards

Kiran

2010-01-07T22:35:00.000+05:30

Hearty welcome to NH blog again. We are in the process of learning something about the developmental history of cardiac drugs. This time, we shall see the development of Calcium Channel blockers.

The 1,4-dihydropyridines have an important role in the human biochemistry. Despite the role it played and the ease with which it can be evaluated, very surprisingly, minimal research had taken place involving this vital compound. The research scientists group of Bayer laboratories learnt this and started working on it. True to their research reputation, they did not leave any stone unturned. Once they had a lead, they tested for its organ efficiency and found a reasonable change in the cardiac output. They focused their energies on the molecular variant and ended up developing and testing more than 2000 analogues! The result was a patent application for a new drug called Nifedipine in the year 1967 by two scientists named Friedrich Bossert and Wulf Vater. They registered the drug for its supposed activity as coronary vasodilator.

With that started the hunt for the actual action of Nifedipine. Over a couple of years, they found that nifedipine acted by blocking calcium channels in the cardiac conduction tissue and in the smooch muscles of blood vessels. Thereby, Nifedipine led to the relaxation of the myocardial tissue and blood vessels. The resultant action was coronary vasodilatation and drop in both systemic and pulmonary vascular resistance. As an obvious result, this should have reduced the afterload and oxygen consumption. Thus, it was a drug with potential benefit in angina with hypertension. With this background, Nifedipine had a grand entry into the markets in 1975.

The major hitch of Nifedipine was its short duration of action. As the popularity of the drug picked up, this issue was to be sorted out. The research groups started the action in two sides.

The first was to develop analogues of nifedipine for longer duration of action. This would have the advantage of a sustained action on blood pressure. The result was the development of felodipine and amlodipine. In the same bargain, some more drugs were developed, which showed a higher specificity for Calcium channels of other organ systems. Nimodipine was one such development with effect on cerebral blood vessels. The advantage was its specificity, in which it did not affect the systemic blood pressure. However, all this new developments would take high levels of research and finances, not to mention the time.

The second approach was more logical and much simpler: creation of a sustained release preparation of Nifedipine. This happened quite fast and was marketed easily. With the popularity Nifedipine enjoyed, it was not difficult to make the presence for the sustained release preparation. However, the research team was aware of the short life of this strategy and had realised the effacement of molecule once the new preparations took over. But the gamble had paid off. The SR preparation effectively bridged the gap and made the constant market for the drug till the newer generation ones took over. Even today, the dihydropyridine analogues enjoy a great market presence in the field.

Compared to the systematic research of Nifedipine, the other calcium channel blocker, Verapamil was more of serendipitous in its invention. It was supposedly a takeoff from the atropine in the field of antispasmodics. In this group came mebeverine, which enjoyed some success. However, the other molecule called verapamil did not have such luck. It was synthesised by the famous Knoll pharma. Its chief of research, Ferdinand Dengel has been credited for its creation and discovery of possible uses. Once the initial trials were over, Dengel realised that the drug was nowhere near the antispasmodic action. What caught his attention was its potential action as coronary dilator. He led the research to take over towards the use in angina. The success of the trials led to the introduction of the drug in the market in 1968. Soon it was realised that the drug can cause depression of conduction activity. This action prompted Albrecht Fleckenstein at the University of Freiburg to evaluate the molecule on those lines. That explained why the drug failed as an agent for smooth muscle relaxation; it was more specific for cardiac muscles! The much more interesting finding of the research was the reversibility of its action by calcium. This led Fleckenstein to assume that the drug was indeed a calcium blocker. Further research showed that the assumption was correct and Verapamil blocked the movement of calcium ions into cardiac cells. It was credited as the first calcium antagonists. As better understanding of the cardiac physiology and the role of calcium channels became apparent, the anti-arrhythmic action of Verapamil was acknowledged in 1970s. Its action as the relaxation agent also became useful in controlling the hypertension, albeit the use is minimal for this purpose at present.

The other calcium channel blocker, Diltiazem has much more serendipitous history! It was supposed to be sought as a psychotropic drug by the Japanese. The Tanabe Seiyaku Company of Japan was working in this regard. After huge number of possible variations in the combination of the medicational research, what they ended up with was Diltiazem! The initial drug was a racemic mixture of many isomers of the drug. The research team ended up segregating each isomer and started testing them. As a part of serendipity, the dextro isomer showed great vasodilator effect. The team decided to conduct the trial for this molecule. It was finally shown to have vasodilatory action simulating papaverine on coronary circulation. Logically, they tested it against calcium ion movement across cardiac muscle membrane. It was found to be a positive blocker! Thus was born the first coronary vasodilator which was actually a calcium antagonist. Later, as a comparison with the other drugs of the group, it was tested for its action on cardiac rhythm. It was positive for that action also! Theoretically, it stood between the Nifedipine and Verapamil, with good qualities of both in the same drug. Its spectrum makes it a desired preparation even today for selective indications.

Next issue may be the last one for the history of drug development. If anyone has any ideas for future posts, please let me know.

On a personal note, we had our departmental “publication meet” recently. As usual, many a write ups are in the long process of preparation. Our mercurial boss got impatient about the delay of her lethargic team. I was advised to give up blogging and to get serious with research. I think it is a good advice for now, as the number of people reading my blog does not make any justice to my effort. I can as well concentrate on writing papers and getting credit for publication! If you find the frequency of the blog posts getting reduced in the future, you know whom to blame!!

ATRETIC TRILEMMA!
How does a restrictive VSD in Tricuspid atresia type II behave? If anyone says as IIb, they are obviously wrong; it becomes subaortic obstruction! It is clearly unlike that of type I. But a beginner needs to understand the finesse of anatomy and should not give feel that a restrictive VSD in Tricuspid atresia II is type b. We had earlier seen a report from outside in which the baby clinically had PAH, but the echo report said Tricuspid atresia IIb. The present baby we saw had a frank narrowed pulmonary valve with sever PS. We recalled the opportunity we had of learning from others mistake! We understood the importance of measuring the size of VSD in such cases and comparing it with aortic annulus. On cath study, this boy had a mean PA pressure of 16mmHg, which was not very much OK for BD Glenn. How about creating a Glenn and doing a PA band at the same time? What is the future of such a manoeuvre? Is there any experience of doing this combo procedure? We could also recall a baby with Tricuspid atresia IC, who had undergone a PA band at age of 4 months and at the age of 2 years, had a PVWP of 17mmHg. Do the post PA bands behave any different from type B physiology in Tricuspid atresia? Does the initial period of high flow change the pressure dynamics in post Glenn any way? Any role of creating a Glenn with PA band tightening here? Please send me your takes on these.

TO BTT OR NOT TO BTT?!
What are the indicators of RV failure in cTGA, VSD, PS physiology? Can the progressively increasing TR be an indicator? We had a 3-year-old baby with cTGA, VSD and PS with more than mild TR. Since the age of the baby is less than the institute acceptance for RV to PA homograft and the baby was symptomatic, it was the choice between BTT shunt Vs an early Senning + Rastelli. How would each of them go about? The chance of getting an optimal sized homograft and the possibilities of repeat procedure were the caveats for Senning + Rastelli. Compared to this, the BTT shunt would increase the RV volume load in a cTGA. Is it OK to load the fragile RV for another year or so? What would be the impact on eventual RV function? Would a failed RV permit us to proceed with any procedure in future? Is there any way of making a vis-to-vis comparison? Any data on this? Please let me know.

CROSSING ARTERIES IN VA DISCORDANCE?
Can cTGA have the great arteries crossing each other? We had a 2-year-old with situs ambiguous, common atrium with ill-defined pulmonary venous connection with interrupted IVC. Now that the atrial morphology could not be defined, the upper floor anatomy was unclear. However, on the echo, the ventricles appeared to have d loop with the VA concordance. One of our team members pointed towards the dilemma of labelling ventricular cavities in AVCD. Hence, if the loop was presumed to be L, then it would be AV concordance with VA discordance! Now, can a discordant VA connection have crossing great arteries? Although I maintained that it is not possible, I was not very sure of my answer, more so in a situation with situs ambiguous! If you have any data on this, please post it.

CARDIAC OR SUPRA?
What is the definition of cardiac TAPVC? The most common answer would be the pulmonary venous confluence draining into coronary sinus or rarely, to directly at SVC – RA junction. We had an infant who presented with features of TAPVC. On echo, we found the pulmonary veins forming a common confluence and going up via the left ascending vertical vein, which entered the persistent Left SVC. There was no bridging innominate vein in this boy. The LSVC came back to heart and opened into the RA via coronary sinus! Technically, it is common confluence opening into CS. Is the variant cardiac or supracardiac? The question is more etymological, as the surgical team had no reservations on surgery, whatever name we called it! But, as a matter of interest, we debated the name. I felt that the cardiac variant should always be in physical link with the surface of heart throughout its entire course and any deviation from it would not be a cardiac variety, more so if it comes with a vertical vein. One of our team members tried to improve my words by saying that the cardiac variant should always be intrapericardial. We would like to get an opinion from the readership. Does the definition involve only the destination or the journey too? Please write your take on it.

MIXED DUAL
Coming back to TAPVC, we often come across mixed TAPVC. Less common but seen variants are the dual drainage, which involves the different pulmonary vein combinations entering RA via separate entry sites. Technically, dual drainage is different from mixed TAPVC. The question is, should they be considered as a separate entity? The traditional Darling’s classification of TAPVC does not mention dual drainage at all. What is the opinion from the surgical clan? Should the naming follow only when the management differs or should it be adherent to the embryological basis? Definitely, the embryological basis of dual drainage is different from that of other variants. What is your take on it? Pen your opinions.

Let me know your opinions on the issues raised or any issues you want to rise! The whole objective of the Blog is to encourage the exchange of opinions on the issues that may not have a straight forward answer. The opinions from your side would be very valuable to lot many for ages. Don’t hesitate to post questions if any genuine problem is bothering you. Let the enlightened readership be your guide to a “Yet to be opened door”. You can either use the comments section or use my email drkiranvs@gmail.com for posting your views and questions. Best of luck.

Regards

Kiran

2009-12-30T23:03:00.001+05:30

Thanks for returning to the NH blog again! We are witnessing the historical development of some of the drugs used in contemporary pediatric cardiology. Hitherto, we have seen histories of Digoxin, Diuretics, Beta-blockers, ACE inhibitors, Prostaglandins and Sildenafil. Let us witness some history of drugs that set our rhythm right: The Antiarrhythmics!

The first victory on the war against malaria came from natives of Peru. The Cinchona bark, also known as Peruvian bark, Jesuits’ powder, or Devil’s bark was probably the first ever medication to be effective against malaria. This medical remedy from the New World (the American continent) caught the attention of Europe around AD 1650s and very soon ended up being the fancy of medical world. This also started an indiscriminate use there on. It did not take much time for the unscrupulous to realise the potential profit making in the Peruvian bark. To beat the competition, many used fake bitter tasting preparations to mimic the Cinchona bark. Worse were the people who used larger doses imagining faster and more effective recovery. This started the downfall of cinchona bark when cases of dangerous relapses (due to fake medicines) and sudden deaths (due to overdose) appeared.

Soon after the introduction of cinchona bark, the German physician Georg Stahl had recognised its action on the heart and subsequently sporadic attempts were made to exploit this. When the side effects of cinchona bark continued to dominate, further attempts to delineate its effect on heart stopped. Celebrity physician of the time, Thomas Sydenham did an extremely careful study on the Cinchona bark. The prevalent belief of the time was a “Peruvian curse” on the cinchona bark. Sydenham argued vehemently against the “curse hypothesis” and proved that the drug was a safe one, provided the discipline of dosage, timing, and duration of treatment were properly taken care of. Despite the best efforts by Sydenham, the scepticism on Cinchona bark continued.

Around 1820, the purified form of quinine was extracted from Cinchona bark and within the next decade, quinine in its purified form was used all over. This gave the opportunity to few physicians around 1850s to prescribe quinine in supranormal doses for management of heart problems, albeit without any scientific evidence. This practice went on for a few years in a selected group of physicians.

As the Dutch had dominated the Cinchona plantations worldwide, availability of Quinine was the best in Netherlands. Not surprisingly, a patient of atrial fibrillations, who visited the famous Dutch cardiologist Karl Wenckebach in 1912, boasted that he himself can control the fibrillations. The surprised cardiologist asked the patient to prove himself. When the patient returned the next day, to the surprise of Wenckebach, his heart rate was well in control.

On enquiry, the patient revealed the secret. He had simply taken quinine to bring his atrial fibrillation under control! What’s missed was the dose the patient had taken which Wenckebach had forgotten to ask. He presumed the regular dose and tried quinine on about a dozen patients. To his annoyance, only one responded favourably. In 1914, Wenckebach brought out his book on cardiac arrhythmias (please note the year: ECG was yet to be used in clinical practice!!)in which he referred to the matter of Quinine’s anti-arrhythmic action just in passing.

Walter Frey was a busy practicing physician in Berlin, Germany. In 1918, he reported about the use of Quinidine in controlling atrial arrhythmias in a prominent Viennese medical journal. He claimed that quinidine was the most effective of the four principal cinchona alkaloids when it came to anti-arrhythmic action. Although Quinidine was supposed to be the least toxic of all the four components it was still a pretty hazardous drug. That made the medical supervision mandatory.

Quinidine was not a new drug. When the cinchona bark was getting purified, many isomers were isolated. Quinidine was one of these isomers that was isolated in 1833 by Henry and Delondre. When tested as antimalarial, it nowhere matched the effect of quinine. The anit-arrhythmic action gave a new lease of life for the once discarded quinidine. From then on, it has never looked back. It can still be used for treatment of supraventricular and ventricular arrhythmias in many occasions.

Procainamide and Its Analogues

A renowned cardiac surgeon of Cleveland, Ohio, Frederick Mautz was a worried man due to the recurrent per and post operative arrhythmias. He had set out to investigate drugs that could be applied directly to the heart surface prevent these arrhythmias. He was logical in assuming that application of local anaesthetics, like procaine, could act as prophylactic medication against these arrhythmias. Added to this, Procaine had already shown to have similar effect on heart.

With this, the animal experiments started. To the excitement of Mautz, procaine indeed showed highly effective action.
With the success of animal experiments, Mautz proposed the use of intrapericardial instillation of procaine for irregular beats of the heart during surgery. Till then, the only option was the use of quinidine which itself had a temperamental action. Procaine was found to be superior to the effects of quinidine.

The problem with procaine was its short duration of action. Added to this, if used repeatedly, the cumulative effect was dangerous to central nervous system. To overcome this, procaine was researched extensively to improve the duration of action, reduce CNS side effects and to improve the cardio-specificity. The result was Procainamide, which is useful till today to treat ventricular arrhythmias on acute basis. When efforts were made to use the drug for long-term, the risk of inducing systemic lupuserythematosus appeared. Hence, it is not for long term therapy.

Efforts were made to improvise the molecule for long term use. In this bargain, the team of French scientists headed by Laville and Justin-Besanc of Laboratoires Delagrange tested 2-chloroprocainamide. Although it had negligible anti-arrhythmic action, it was found to be a very effective antiemetic on oral administration. This was further improved to create metoclopramide.

In the next post, we shall see the development of another group of drugs. Just before that, let me tell you that I am about to finish this series on the “developmental history” of drugs used in Pediatric Cardiology. I need some ideas for the new series. If anyone can give me some ideas for the new series, it would be very helpful. Please note that the responsibility of the research on your ideas would be entirely mine! I would not disturb you for any further help, unless you wish to provide me some. However, the credit for the idea will be fully acknowledged in the blog with the full name of the contributor!!

On a personal note, the New Year is fast approaching. Our team is depleted as of now! Consumption of remaining leaves or the wretched climate of Bangalore causing respiratory problems has taken the toll on the rest!! With half the team doing more than full work, the time available for decent academics and discussions get progressively shorter. Hope, we shall get back to full strength soon.

VENTRICULIZATION OF PAs
What if the cath tracing of PA mimic that of RV? We had a 4-year-old who had undergone ICR for TOF with transannular patching a couple of years back. He came back with an aneurysmally dilated RVOT. On cath, PA tracing was as described above. Unless we see the location of catheter on screen, we cannot make out the PA entry on tracing. Traditionally, homograft repair is considered as the surgical option. However, our surgical team felt that a reduction plasty of RVOT can be done. Is the impingement of this dilated RVOT on LPA very common? If any data with anyone, please let me know.

AMPULLA Vs DUCTAL CONSTRICTION
What should be the ideal device for a bizarre PDA? We used a regular PDA device in a bizarre PDA in a 34-year-old. The larger side of device sat nicely in the ampulla and before the release, it slipped into the duct and found its home at one of the constrictions of the duct. Before we could make any other change, the device seemed to settle well there. We decided to leave the device as it is. What might be the possible complications? Is the muscular duct as good as the ampulla for accommodating the device? Any experiences in this regard? Please comment your ideas on it.

NOT HOLMES’, NOT JOUBERT’S
The DILV with NRGA would be a Holmes’ heart; the same with malposed GA would be a Joubert’s heart. How about a DILV with side-by-side great arteries? We happened to see one like this. This baby had a classical DILV, but with PA from LV and Aorta from OC. On short axis, they had a side-by- side relationship. Is there a name for such a combo? How common are these entities? If you have seen one, please tell me.

EBSTEINS IN CTGA
Is the TV anatomy in Ebsteins with AV concordance any different from Ebsteins with AV-VA discordance? We know that physiologically, they connect to different great arteries and different pressures. But, anatomically, is the displacement or histology any different? Does it translate to any difference in surgical approach and outcome? What does the surgical team opine about this? We had one of our senior surgeons mentioning the practical difficulties of treating a CTGA with Ebsteins. If there are issues, can we take any measures to ensure better outcome in the post-surgical period? If any research has been made on this which you have come across, please send it to us.

SINGLE DILEMMA
We often come across infants with Single Ventricle physiology with a moderate PS. Many times these babies saturate around 75-80% on baseline. What can be done for them? The PS would not be sufficient to enable BD Glenn. The saturations do not allow PA tightening. BT shunt would be negative for future Glenn. How should we be going about? Please drop in your suggestions.

TRANSITIONAL CYANOSIS
We saw a 2-year-old with Transitional AVCD with common atrium and uncertain pulmonary venous drainage. The pulmonary veins were draining into the roof of common atrium all around. The IVC was interrupted and there was bilateral SVC. There was an inevitable mixing at the atrial level, as for any other common atrium. However, this baby had mild cyanosis and clubbing with the saturation of 80%. Such picture does not occur in regular cases of common atria. How does the streaming affect the situation? We are planning to establish the correct anatomy of pulmonary veins in this baby with a cardiac CT. Can the interrupted IVC explain this picture? If you have come across such a picture, please tell me about your observations.

Pen in your comments. Use my email drkiranvs@gmail.com if you find any problem posting your comments.

Happy, prosperous and eventful 2010 to all of you

Regards

Kiran