Tuesday, September 28, 2010

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

Friday, September 17, 2010

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

Tuesday, September 14, 2010

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

Wednesday, September 8, 2010

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