Sunday, September 13, 2009

Welcome back to this wonderful journey into the History of Pediatric Cardiology.

In the spirit of writing history, we started with the works of masters, followed by biographical sketches and lets end with celebrating the events. Any Art or Science culminates to its glory when it ceases to be Individual and dwells upon the Teamwork, with few sung and numerous unsung Heroes. (As for Pediatric Cardiology, Heroines overtake Heroes by huge number!!)

We are in the year AD 1938, when the world witnessed something to remember. The concept of the era was aptly described in AD 1896 by a person of phenomenal respect, Dr Stephan Paget. He had said, "Surgery of the heart has probably reached the limits set by Nature to all surgery. No method, no new discovery, can overcome the natural difficulties that attend a wound of the heart."

Just about one and a half year before, on March 6, 1937, Dr John Streider at the Massachusetts General Hospital had successfully interrupted a ductus for the first time in history. But his choice of patient selection was probably wrong. His idea was to save a septic patient, but the patient died on the fourth postoperative day. At autopsy, vegetations filled the pulmonary artery down to the valve. This heroism went unsung by the medical fraternity and Dr Streider retired to his regular surgical practice.

On 16th August, AD 1938, the boss of Surgical department in Boston Children’s Hospital was on leave, leaving the department to the care of his junior assistant, Dr Robert Gross, a dynamic and equally arrogant young surgeon. Dr Hubbard was a friend of Dr Gross who ran the show at the pediatric department of same institute. On a tea break, Dr Hubbard mentioned a child with Ductus in the ward. On a fine moment, he suggested Dr Gross to operate on the child. A hesitant Dr Gross agreed after much cajoling with the possible assurance that, if something goes wrong, his boss would not be informed and involved! He probably did not oversee that the world would remember him and not his boss after that moment!!

So, this 7-year-old girl, Lorraine Sweeny, with dyspnea after moderate exercise, who was ferried to OT with a doll in her hand, was operated by Dr. Gross. He described the ductus as 7 to 8 mm in diameter and 5 to 6 mm in length in his operative notes. He used a no. 8 braided silk tie around the ductus with an aneurysm needle, and occluded the vessel temporarily for a 3-minute observation. During this time, blood pressure rose from 100/35 mmHg to 125/90 mmHg. He wrote, "Since there was no embarrassment of the circulation, it was decided to ligate the ductus permanently." The patient made an uneventful recovery and History was created. The case was reported in JAMA in AD 1939 and created a sensation in the surgical circles. Dr Gross became a big name.

Few hours from Boston, Dr Taussig was active in Baltimore, discussing the possible causes of levels of cyanosis in blue babies. Her astute observation had taught her that blue children with continuous murmur were less cyanotic than those with systolic or no murmurs. She had correctly reasoned that the murmur was due to the continuous flow of blood from vessels that left the aorta and anastomosed with the pulmonary artery or its branches, thereby increasing pulmonary blood flow. Which simply meant that any measure to increase the pulmonary blood flow would make the blue babies better! When Dr Gross’s success was celebrated for technique and results, the brilliant thinking of Dr Taussig went lateral. If a surgeon could tie off a patent ductus, could he also create one?

This question turned out to be a dream that could not make her sleep! She wanted an answer and in positive. She drove all the way from Baltimore to Boston to meet Dr Gross, who was now used to adulation for his work. But the lady with a huge hearing aid stood in front of him and instead of congratulating, asked a sharp question, “Can you create a duct?”

Dr Gross was taken aback. Neither he expected the question, nor he knew if it was possible. The surgeon’s arrogance momentarily filled him. He curtly said that he was in the business of closing a patent ductus, not in creating one, and left the room.

Dr Taussig was disappointed, but in no way gave up. She decided to put a reason to the unyielding surgeon, but he was equally firm. When she decided to give him sometime and try again, the Second World War broke in. Doctors, as the government decided, had better works to do than experimenting newer techniques and research. This ended up putting Dr Taussig into back gear. She never last her touch with her dream and was in the habit of asking many surgeons the same question. Her deafness had made her lose the sense of pitch and she would ask the question in a very high note, annoying any surgeon who would see more of a tease than a reason in her question!

“When something is wished from the heart, the entire universe conspires to conjure it for you” is a popular Bollywood quote! The heartfelt wish of Dr Taussing came searching for at her home, the Johns Hopkins, in the form of Dr Alfred Blalock and his phenomenally skilled and gifted surgical assistant, Vivien Thomas (who was honored with a Doctorate in later years) in the year AD 1942. How and why this combination worked and created history would be the content of my next post.

(Ref: Graybiel A, Strieder JW, Boyer NH: An attempt to obliterate the patent ductus in a patient with subacute endarteritis. Am Heart J 1938; 15:621.

Gross RE, Hubbard JH: Surgical ligation of a patent ductus arteriosus: report of first successful case. JAMA 1939; 112:729)

On a personal note, one of the earliest followers of the blog informed me that he finds it convenient to skip the history part and come directly to the segment that starts with, “On a personal note”! I wish to know if I am very heavy on the history part. I have always tried to make it more “storylike” than “textbooklike”. If anyone has any suggestions in changing the history part to more pleasing and acceptable, please let me know. However, I had huge solace when Dr Shweta Nathani (who was on leave for a month for her marriage) told me that she read all the missed out posts in a single go and found it very interesting, I was really happy. I should take this opportunity to congratulate Dr Shweta and Dr Yogesh Bhakru on their marriage and we all hereby wish them for a greater glory in life.

Has anyone come across Left Ventricular aneurysm in a newborn? What is the natural history of such a lesion? We had a newborn with aneurysmal LV in its free wall. The overall function of LV was reasonable and the baby was asymptomatic. The neonatologist had noticed an abnormal radiograph and had referred the baby to us. Coronaries on echo appeared normal and we even contemplated a cath study for coronaries. However, the asypmtomatic nature of the baby put us on the backfoot. We have asked for a follow up after a month and earlier if any symptoms develop. I will post the follow up SOS. If anyone has any data on this, please let me know.

We had a 17-year-old girl of single ventricle physiology. She had bilateral SVC, but had already undergone unilateral BD Glenn outside few years back. On cath, we found a bridging vein, which deflected about 30% of LSVC flow to Glenn circuit. Her McGoon ratio was 1.6 (largely due to small LPA), making her unsuitable for Fontan. How should we deal with LSVC now? Should we put LSVC to LPA, with expected promotion in its growth, or just close the distal LSVC to enable the entire flow to pass through bridging vein and into Glenn circuit? This girl is symptomatic and needs some intervention. Our senior surgeons feel that the surgical effort should translate to a beneficial quantum to the patient. Would dealing with LSVC serve the purpose in a 17-year-old? Should we just risk a Fontan completion? Few questions are answered only on a retrospect.

In a DORV, when the subarterial conal tissue comes in the way of routing VSD to Aorta, can it be resected? I have heard surgeons arguing that there is no such thing as conal tissue resection. At the same time, I have also seen children with such problems getting the 2-pump repair done successfully. The disparity between a 2D image of Pediatric Cardiologist and a 3D vision of surgeon on the table makes all the difference. But, how to formulate a preop plan? Not every patient can reach the OT table with Plan B. Any personal experiences by anyone in this regard? Can 3D Echo imaging be of any help? Please let me know.

I had seen a 2-month-old girl with obstructed TAPVC, DORV, side-by-side great vessels and a large VSD with sever PAH. The side-by-side relation is always puzzling on whether a straightforward VSD closure can do or if it needs a tunneling. If tunneling is not possible, can we do an arterial switch? If all these appear too complex, should we just repair only TAPVC and do a PA banding? Surgeon would ideally seek a clear plan and we have insufficient data. We sometimes feel inadequate to deal with some lesions.

How many of us have come across a Partial AV canal defect with Transposition of Great Arteries?! We had such a child aged 2 months. The LV was not prepared. Moreover, it was Situs Inversus, Dextrocardia! The option of a 2-stage rapid switch was challenged by the Surgeons themselves. The rarity of the lesion complex was unwelcoming for any surgeon. Earlier, we had seen a Transitional AV canal defect with Corrected TGA. Our senior surgeon, Dr Shekar Rao seriously joked that we should report the case in Ripley’s Believe it or Not Journal!!

We have a 13-year-old girl, who has undergone BD Glenn 8 years back for DORV, VSD, d-malposed arteries and PS. Now, she presented with hemoptysis. The first obvious culprit was collateral development. When we reviewed her echo, we thought that her VSD was routable. As taking down BD shunt is tough now, we are contemplating one-and-a-half pump repair with VSD closure and RV to PA conduit. Incidentally, she tested positive for Tuberculosis and is being treated for it. Does the conduit repair come in the way of Glenn function? If anyone had any similar experience, please let me know.

It is very painful to see the team getting ripped off. Also, inclusion of any hitherto unknown new member to the team is also a substrate for apprehension. I quoted Stephan Covey (7 Habits of Highly Effective People) that the teamwork sums up to more than the individual components. As the corollary goes, when a member of the team leaves, we lose more than his part. Such logics are very difficult to convey to the management guys. They just look out for “replacement” without understanding the implications and wound on the tem spirit. Retaining talent and dedication is an important aspect of building an institute. But it may not be very easy to realize the truth unless the management guys are a part of the team.

Any suggestions and comments are welcome. If you face any difficulty in posting them, please feel free to mail the same to drkiranvs@gmail.com I shall post them on your behalf.

Regards

Kiran

Sunday, September 6, 2009

Welcome back to the fascinating journey called History of Pediatric Cardiology.

AD 1898; the year Dr Maude Abbott joined her job in McGill, another person was born in Cambridge, Massachusetts. This girl was to become the “Mother of Pediatric Cardiology” in future. Despite losing her loving mother by the age of 11 years to tuberculosis, despite suffering from dyslexia as a child and rejected by school, despite suffering from Pertusis and having partially lost the hearing ability in childhood, she made it to college education. Thanks to her Father’s interest in teaching her personally and inspiration from her Grandfather, who was an acclaimed Physician.

She overcame all the difficulties in personal life to earn a seat at Harvard’s School of Public Health and got special permission to take courses at Harvard’s Medical School and at Boston University. At that time, Harvard was yet to open the doors of its medical school to Women. While studying at Harvard, an Anatomy professor suggested her to specialize in cardiac research and to apply at Johns Hopkins University at Baltimore, Maryland, one of the few American medical schools that accepted women. She followed his advice and went to pursue her interest in cardiac research. She graduated from Hopkins in AD 1927, and served as a fellow in cardiology at Johns Hopkins Hospital for the next year, followed by a two-year pediatrics internship.

By the time of graduation from Hopkins, she had lost her hearing almost completely and relied on lip-reading and hearing aids for the rest of her career. Some of her innovations in Pediatric Cardiology have been attributed to her ability to distinguish the rhythms of normal and damaged hearts by touch, rather than by sound. It was always said that she auscultated from her fingertips. (One of our fellows at Narayana Hrudayalaya always quoted this and teased us on our emphasis on development of auscultatory skills!)

Thanks to the mentorship of Johns Hopkins Chairman, Mr Edwards Park, in AD 1930 she was appointed head of the Children's Heart Clinic at the Johns Hopkins Hospital pediatric unit, the Harriet Lane Home. He encouraged her to concentrate not only on rheumatic fever, the big problem, but also to learn about congenital heart disease. Here, she worked until her retirement in AD 1963.

That’s correct. We are referring to Dr Helen Brooke Taussig. The wonderful lady who contributed so much to Pediatric Cardiology that we should be indebting our careers in this field to her.

Dr. Taussig was meticulous to the core. She had the habit of recording her findings in each patient. She realized that certain malformations repeated with characteristic and recognizable patterns. The concept of ‘Pattern Recognition’ in Pediatric Cardiology was her contribution. She correlated her observations with the anatomy at postmortem examination. Soon she was able to make clinically an accurate anatomic and physiologic diagnosis, using the tools of history taking, physical examination, electrocardiogram, fluoroscopy and X-ray. She paid particular attention to pulmonary vascular markings, whether increased or decreased. Despite all her efforts, she could not help the children in prolonging their life; forget curing their heart problems.

The 4th decade of 20th century was a crucial one. It was seeing new waves in the front of cardiac treatment. The new age surgeons were bold enough to challenge the age-old concept of “not to touch the heart”. Dr Maude Abbott had brought about a revolution by her ‘Atlas of Congenital cardiac Disease’. Dr Taussig visited Dr Abbott and was fascinate by the visit. The atlas served as an important treatise to clarify some of the symptomatology that hitherto perplexed the physicians. It had also classified malformations according to the presence or absence of cyanosis.

Dr. Taussig went a step ahead. Instead of accepting the Atlas as the final compendium, she started developing further leads from it! She utilized the barium swallow technique of Chest Radiography to identify a right aortic arch. This information became very practical later for creation of BTT Shunts, for, it utilized the subclavian artery on the side opposite to arch sidedness. But on the personal front, Dr Taussig had lost her hearing completely and used a huge hearing aid, which hung from a cord around her neck. People ridiculed her appearance, but it was good that either she did not hear them or cared the least about them. She was so committed to her work that she had no time for such trifles.

Let us stop at this point for the week. It is AD 1938 we are talking and Pediatric Cardiology was about to change forever. It is no longer the saga of a single person, but the beginning of a solid team effort. Let us continue the saga next time.

I could not go to the camp at the town of Ranibennur due to a heavy bout of Sinusitis. The same is the cause for the delay in this post.

On a personal note, the preceding week gave us many learning experiences. I happened to see a one-year-old child with Situs Inversus, Dextrocardia, cTGA, Ebstein’s anomaly of Tricuspid Valve, large VSD, mild subvalvar PS and mild PR. The RV cavity appeared good enough to sustain the pulmonary circulation. But the child was totally asymptomatic, largely because of balanced physiology it had at present. One can understand the complex anatomy and the apprehension of surgical team to touch this baby. The prospect of clearing the LVOT obstruction completely to enable a double switch and repair of Ebstein Tricuspid valve puts too many caveats on a successful outcome. Making the moderately educated parents understand the anatomy of their child was such a complex task, making them understand the complexity of surgical procedure was near impossible, despite all the props we used! The idea making a parent understand their normal looking baby may have such a high risk for surgical correction is a challenge indeed. Finally, the parents sought some time to express their opinion and are yet to come back. Such anatomies not only put our subject skills into test, they also put our communicative skills to higher challenges!

We discussed the utility of our diagnostic modalities in providing the data for surgical decisions. We had an 8-year-old boy with Tricuspid atresia IB, with a McGoon ratio of 1.2 on cath. The echo showed the same to be 1.6! We know that on echo we do not always measure the prebranching PA diameter, as it is not always possible. However, the surgical team argued that the PA are underfilled on cath and the actual McGoon may be far greater than what we have measured. As the child is definitely not OK for Fontan correction, we decided to go for either a BD Glenn or a BTT shunt as the final palliation. The surgical team favoured the former and we, the latter. On the table, the PA were indeed small and the surgeons came out with a BTT shunt. Our argument was incidentally correct and we agree that the decision could have easily gone the other way. But this incident opened up our dilemmas in decision making despite the completion of presurgical analysis. How are the other centers across the country and world making these decisions? If anyone has the data, please let us know.

What is the natural history of an untreated unicuspid aortic valve? Our senior surgeons feel that it is not much. To the surprise of everyone, we saw a 13-year-old girl with unicuspid aortic valve with severe stenosis. We felt that the ballooning of such a valve may not be ideal. Surgeons asked us to try a balloon as not many such scenarios are seen in history! If anything goes wrong, our surgical team is competent enough to take care. Any inputs on this from anyone?

Another 16-year-old boy probably had the anatomy of the month! He had a concordant Viscro atrial situs and atrio-ventriculat situs with discordant AV connections. But aorta (which came out of RV) was posterior and to the right and MPA (which sprung from LV) was anterior and to the left. This corresponded to the Normal Great artery relationship. So, it is TGA with NRGA {S,D,S}. Any such incidents seen by anyone? It was totally new for us; we are yet to understand the embryological basis of such a defect.

If LA mean is 6 mmHg and PA wedge is 17 mmHg in a child with ASD, what is the inference? This child underwent cath study to explain unusual TR gradients on Echo. We suspected pulmonary venous pathology. Dr Sunita correctly pointed out that PA wedge of both branch PAs should be taken. However, a review echo did not show any obvious pulmonary venous pathology. We contemplate a cardiac CT now.

If anyone seeks operability in a common atrium with interrupted IVC, how to ascertain the Venous saturations? The interrupted IVC, which does not receive the hepatic blood, would show a higher saturation. Doesn’t the Qp calculations and thereby PVR go wrong? Can the borderline operability in these cases be decisive on cath? Any correction factors? We unfortunately lost such a kid after surgery. Were we wrong in analysing the cath data? Our cath showed a low Qp/Qs but also, a low PVR. The mathematics really eluded me. If anyone has any experience in this regard, please let us know.

Our department is undergoing major transformation. The present ‘market’ for Pediatric cardiologists in India is bright, which is good for the Doctors. On the lower side, low paying institutes like us will lose out good people and would find it difficult to get new ones. We are in such a situation at present. Just hoping and praying that we would come out of it further unscathed. Yes, pure hope and nothing else!!!

I would like to introduce our new follower. He calls himself docspike. He operates a blog by name Yukon Ho! (An obvious Calvin and Hobbes lover). He is Dr Ravichandra Karkal, from a small town called Ilakal in Karnataka. Ilakal is famous for its typical traditional sarees. Welcome aboard, Dr Ravichandra. We hope to see your contributions in our blog soon.

I hope the readership is increasing. However, I need more replies, responses and interaction. If you find it difficult to post in the blog, please send them to my email id drkiranvs@gmail.com I shall post them on your behalf.

Regards

Kiran