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

This post would be the last in this series of Historical write ups. The history of Pediatric Cardiac Surgery, Pediatric Cardiology and Adult Cardiology gets quite intricate from the mid 20th century. It may not be possible to isolate one from the other. So, we shall find few other ways of getting into historical aspects. It may be fitting to trace the origins of Pediatric Caridiac training in the present post.

I was asked a couple of times on my emphasis on BTT shunt and not on the history of development of other ways of treating TOF. The idea was to show how the Pediatric Cardiac training developed with the development of BTT shunt. However, it may be prudent to answer the others too!!

The first world war had decimated the earliest efforts in the treatment of TOF. French surgeons were working on attempts to relieve pulmonary stenosis in Paris as early as 1913–14. Doyen, Tuffier and Carrel were working independently on it; Unfortunately, once the efforts were broken, further operations to relieve right ventricular outflow tract obstruction were not undertaken until after World War II.

The surgical historian Raymond Hurt describes the other side of TOF repair with great elan. In his words, “The first successful pulmonary valvotomy was performed by Holmes Sellors (London) on December 4, 1947 on a 20-year-old patient with tetralogy of Fallot. It has been intended to perform a Blalock procedure but at operation the firm structure of a stenosed pulmonary valve was felt which protruded into the pulmonary artery with each heart beat. With typical great aplomb he asked for a tenotomy knife and this was introduced through the infundibulum of the right ventricle and the stenosed pulmonary valve cut in two directions. Immediately the cyanosis almost disappeared. The report of this operation appeared on June 26, 1948, two weeks after Brock’s report of his own later three successful operations early in 1948.

However, it was Brock (London) who was the main protagonist for the direct operation of pulmonary valvotomy and he thought that ‘‘relief by direct attack must be our goal,’’ in the belief that there were two inherent problems associated with the Blalock-Taussig operation for tetralogy of Fallot: the likely failure of the shunt to enlarge as the child grew older, and concern about the possible harmful effect of the increased blood flow on the pulmonary circulation. In 1948 he designed a valvulotome to cut the stenosed valve, an expanding dilator to split the valve, and an infundibular punch to resect the thickened infundibular muscle. He reported the successful use of these instruments in 1950 and wrote that ‘we are in large part recompensed for the long and difficult hours by seeing the almost miraculous change in the children themselves and in witnessing the joy and relief of the parents when they see their children running about happily and without effort like other children.’

The Brock procedure was immediately adopted in the United States and in Europe for those patients with pulmonary stenosis and an intact interventricular septum, following which the introduction of hypothermia and subsequently extracorporeal circulation in the mid 1950s enabled an open and therefore more precise operation on the abnormality to be performed.

For the treatment of tetralogy of Fallot, it was never established whether the direct operation (Brock procedure) was preferable to an indirect anastomotic operation—as Lillehei (Minneapolis) introduced total correction of the abnormality by an open technique, first in 1955 by controlled cross-circulation, and then in 1956 by heart-lung bypass.”

Let us get back to the post BTT shunt era. The success of consecutive BTT shunts led to the publication of the article, “The surgical treatment of malformations of the heart in which there is pulmonary stenosis or atresia” in the year 1945 in JAMA by Drs Blalock and Taussig. This led to the influx of cyanotic patients from all over the world to the team at Hopkins. The influx was too heavy for Dr Taussig to handle alone. The new set of patients needing attention called for assistants. Hence, Dr. Taussig devised a novel way of dealing with the crisis by appointing interested young doctors as fellows and thereby started an establishment called training program in Pediatric Cardiology.

The grace with which Dr Taussig and Dr Blalock divided responsibilities between their teams is worth understanding. It would go something like this: Her team for the diagnosis and recommendation for surgery, both the teams for the decision at a joint conference to advise the operation on each patient, his team for the operation, they for postoperative care in the hospital, and her team for the long-term follow-up. Both teamswillingly gave their time for talks at medical and surgical meetings and they accommodated the many doctors who came to observe the coordination of pediatric cardiac and surgical care. Dr. Taussig analyzed not only the many successes of the operation but also the failures. This module is the recipe for successful Pediatric Cardiology team even for today.

The catheterization techniques developed in the subsequent decade changed the morphology of Pediatric Cardiology. Wider recognition led to the development of the first subspecialty section of American Academy of Pediatrics in 1957, that of Pediatric Cardiology. It started with 47 members and is ever growing. Four years later, in 1961, came the board examination for certification and the establishment of training programs in this field with high standards. On similar grounds, International development of Pediatric Cardiology specialty began in 1964 when the Association of European Paediatric Cardiologists was established, which had 57 members from Europe and 17 from the United States. Thereby, the first World Congress of Pediatric Cardiology was held in London in 1980 with Dr. Jane Somerville in charge of the planning committee. The rest, as they don’t say, is no longer history!!
With this, I intend to end the history component of the posts. However, if something very interesting is found, few snippets may return. If anyone thinks a bright idea for the contents of future posts, please send your suggestions either in comments or by email to drkiranvs@gmail.com or to drkiran.vs@hrudayalaya.com

On a personal note, the turmoil at the NH Pediatric Cardiology seems to be settling! Many of us expected this, although each probably had their own reasons to come to conclusion. All might have summed up and we expect to find a smooth show again. Thanks to all those who directly or indirectly helped to this effect.

We had a couple of indecisive moments in patient management recently. How much of significance be given to the history by parents who are so ignorant that they might not have understood simple questions like the extent of effort tolerance and symptoms? Our folk are sometimes so ignorant (?) that they don’t understand the value of history given by them and how it affects the decision making. We had a 9-year-old boy with severe aortic regurgitation. During initial interview, the parents gave the history of effort intolerance of NYHA Class 2 to 3. He was admitted for surgical repair in the ward. However, we found the boy happily playing in the ward with other children of his age group. We interviewed the parents again and this time, their history was that the boy had no significant symptoms! This put us into a lot of turmoil. If the boy is asymptomatic, are we justified in doing any surgery on him? Which of the two versions of the parents is correct? Is there a fool-proof way of decision making in such grey zones? Fortunately, our senior consultant, Dr PV Suresh came up with the idea of doing a treadmill test for the boy. We monitored the entire procedure and our patient could achieve only 5 mets. So, our decision for sending the boy for surgery was thereby justified. I felt that the suggestion of treadmill was a brilliant one and we should probably make use of this test much more frequently in pediatric cardiology, more so when the decisions are based on history given by such parents who themselves are not convinced of what they are talking! If anyone had any experiences in such issues, please tell me how you solved it.

How do you define a truncus arteriosus? We use a combination of single semilunar valve with subtruncal VSD with truncal origin of pulmonary circulation proximal to the origin of neck vessels. If all these criteria are met, a diagnosis of truncus is made. But imagine an infant with long segment Pulmonary atresia with subaortic VSD with Aortopulmonary window with confluent branch PAs! How would one differentiate this from Truncus during Echo? Our surgeons found this on the table, closed the AP window and created a BTT shunt. We plan a future Rastelli procedure. Now the question: Why not do the same for all infants with Truncus where we have or can have unifocalised PA circulation? Isolate the Pulmonary circulation from trunk, create a BTT shunt and let the child grow to receive Rastelli in future. What is wrong in doing it? In fact, we have reported a case of aortic atresia with AP window in Cardiology in the Young. However, the present was a new brain wave!

How does the location of VSD decide the symptomatology of children with a ventricular level mixing lesions? We have seen infants with TGA with restrictive PFO with outlet VSDs where the mixing is so inadequate that the baby goes for a balloon atrial septostomy. However, if the VSD is inlet or has an inlet extension, the mixing is so good that the baby would come quite stable. It seems to have an anatomical basis, but finding inlet VSDs with TGA are not that common. If you had any experiences in this, please let me know.

Has anyone seen {S,D,I}?! I mean, an I malposed great arteries in case of normal situs and D looped ventricles? This baby I saw had a VSD and PS and saturated about 70% in room air. There were no windows for short axis and what I saw in other views would not convince me to say that it is {S,D,I}! I had to seek professional expertise of Dr Sejal Shah, who concluded the combination! It was tough to believe as none of us had seen this combination earlier. Are such cases reported? Please let me know your experiences.

NH is restarting the 3-monthly newsletter called Hrudaya. I shall post something interesting from it in future posts. He we get some great experiences from “bigs” of this field!

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