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

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