Sunday, October 18, 2009

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

Sunday, October 4, 2009

In our voyage on History of Pediatric Cardiology, we have coursed till the prologue to a historical moment called “First BTT shunt”. The moment needs to be savored a bit for the completeness of experience. So, some background to the moment:

What made Dr Blalock an ideal person to create first BTT shunt is also worth knowing. The serendipity was more than an extraordinary coincidence. In his own words, Dr Blalock was a person with his perspectives in Future than in Past. Since 3rd decade of 20th century, Dr.Blalock was working on experimental shock. He used to utilize simplest of the experimental laboratory. The instrumentation and equipment were minimal. Dr Blalock had found Vivien Thomas by then in strange situations. Dr Thomas was a high school graduate from Nashville. Despite brilliance and hard work, he was unable to continue his studies in college due to great financial depression of USA, which saw the closure of the Bank in which he had saved all his painfully earned pennies. He went to work as a carpenter’s assistant and was so skillful that he could create new tools to make the job less troublesome and more creative. In AD 1930 he came to work at Vanderbilt and became a laboratory technician for Dr Blalock. They formed a professional relationship that lasted until Blalock’s death. Blalock’s incredibly productive laboratory work during the 1930s is largely attributed to hard work and meticulous record keeping of Dr Thomas. Everyone who visited the lab noticed his attention to detail. Many of these visitors became great names in the field and always applauded Dr Thomas and held him in high regard later.

The team of Drs Blalock and Thomas had been working on Pulmonary hypertension modules for a long time. For this, they turned the subclavian artery of dogs and connected them to ipsilateral Pulmonary artery. This flooded the lungs and finally caused pulmonary hypertension. They were successful in about 200 such experiments on dogs.

Success of PDA ligation from Boston was so catching that every surgeon in US of A was trying to do it. Even Dr Blalock was not left behind. In AD 1940, he had successfully closed PDA in a patient. It gave him the insight for future triumph. However, by the onset of 5th decade of 20th century, the competition on surgical repair of coarctation had begun and was on a high between American and European continents. Surgeons across Atlantic tried to outbeat their counterparts. So, literally every gathering in which surgeons met had a discussion on Coarctation repair. One such gathering in AD 1942 in Johns Hopkins had Dr Blalock discussing his ideas on coarctation repair with Dr George Duncun and Dr Edwards Park. They were talking on the practical difficulties of surgery in which coarctation segment was excised, left subclavian artery was divided, and the proximal end was turned down and anastomosed end-to-end to the descending thoracic aorta. For this, Dr Park said, “Could you not use the carotid artery as a bypass? It is a long, straight artery and there are four vessels to the brain. Wouldn’t it be possible to turn the carotid artery down and anastomose it to the aorta below the coarctation?”

None of them were probably aware that Dr Taussig with her huge hearing aid was standing close enough to hear a part of the conversation. She barged in to ask, “If you could put the carotid artery into the descending aorta, couldn’t you put the subclavian artery into the pulmonary artery?”

Dr Thomas, who was in the room as a party assistant reminded Dr Blalock of their dog experiments to create pulmonary hypertension. At that moment, impressed with the honesty, concern and commitment of Dr Taussig towards the blue babies, Dr Blalock agreed to give up the competition on coarctation repair (which, he was on the verge of winning) and take up the challenge of creating shunts. But, he was not an easy cake. Despite the 200 and odd successes they had in dog experiments, Dr Blalock insisted that an animal model be created mimicking a cyanotic heart condition, and once this was achieved, they determined whether or not experimental shunts, such as had been performed years ago in an attempt to produce pulmonary hypertension, would in fact reduce cyanosis and the polycythemia associated with it. Only when he was satisfied with the animal model, he decided to do it on a live patient. However, he insisted that the first patient should be so handicapped that the risk of the new surgical technique was justified. A hunt for such a patient started in the wards of Johns Hopkins.

The 6-year-old Eileen Saxon qualified for the surgery. She was frail, had many hypercyanotic spells and deeply cyanosed. On 24th of November, 1944, history was created in the OT of Johns Hopkins, Baltimore when first BTT shunt was created by turning the subclavian artery to join Pulmonary artery. In the operating room that day Vivien Thomas stood behind Dr Blalock, and Dr Taussig positioned herself at the head of the table by Dr Merrel Harmel, the anesthesiologist. When the anastomosis was completed and the clamps released, Dr Taussig exclaimed with delight that Eileen had a “lovely pink color”. Dr Denton Cooley and Dr Mary Engle (both became big names in the field) stood as surgical assistants to witness the making of history. Although Eileen did not make it to many days, her brief stint of survival gave hopes to medical community and thousands of such patients to venture into the future of Cardiac surgery.

In the following year at Johns Hopkins Medical and Surgical Association annual meeting, the auditorium was packed with faculty and students. Dr Blalock described the operation and Dr Taussig brought in the first five surviving postoperative children with their smiling faces and normal pink color. She told how before the operation they could not walk across a room without stopping to squat down to rest and catch their breath.

We shall see how the world of Pediatric Cardiology changed with this in our next post. Just as a postscript note, Dr Blalock lost the race of Coarctation repair to his European counterparts. On October 10, 1944, about a month prior to Dr Blalock’s surgery on Eileen, Dr Craford and Dr Nylin, at the Karolinska Hospital in Sweden, successfully repaired a coarctation of the aorta. Dr Blalock would have won this race easily if he had pursued Coarctation repair. But, he achieved a lot bigger and perhaps, a lot better.

On a personal note, this post comes after a long delay, largely due to some turmoil in our team. Close to the resignation of Dr Amit Misri from the team, Dr Sunita, our dynamic head, decided to quit from the administrative post as the head of the team. Her decision was probably long pending. She was frustrated with the nutheads of the non-medical management personnel who run the show. The chaos that followed her decision was so intense that we are yet to recoup! It was only after her decision that we realised how much work was done by her for the team. Even after two weeks, we are yet to realise the amount of her work we have to distribute for smooth functioning, let alone doing it successfully!

Added to it, another cause of delay was my vacation. I went to a small village in Kerala called Thekkady. It is located in the district Idukki, at a tehsil called Kumily. It is adjacent to a large conserved forest by name Periyar. The huge lake in the forest offers boating facilitated by the Kerala Forest department and Kerala tourism. We were shocked to see the glaring absence of lifeguards, life-jackets and necessary equipments for dealing with any casuality. When we questioned the authorities there, they casually replied that nothing of any emergency has happened yet. Nevertheless, for having paid a huge amount for the trip, we completed it. Within four days, the same boat we travelled by capsized, killing 40 odd people! It was a mere miracle that people have survived such a mishap for long, as this could have happened anytime to anyone, including me four days back.

Kerala is one of the highest earners in the country for tourism. Their fees for any event are atleat double of what is charged in rest of the country for the same facility. They also boast of the highest literacy and public awareness. If the cost of life is so cheap in such a place, imagine what the state of affairs in other states in our country. I wrote about the tourist spots our country can offer sometime back. I should add that the socio-political scenario of this country is not mature for any tourism. When government runs the show, the administrators are so callous that the entire show stinks at its worst. When a private person runs it, the only goal would be to loot the helpless tourist, as the contractor has paid huge kickbacks to the Government official, which he wants to recover it at the earliest, irrespective of the means. There needs to be a model that is clean, safe, within budget of common citizen and transparent. Huge income awaits the successful entrepreneur. But, the higher up persons in the power cannot do it for many reasons. It can be ignorance, lack of will power, lack of motivation, inertia or anything imaginable. Lot of people can realize the truth. But in a country where power lies with people because of Family legacy, caste and creed, social contacts, sycophancy, money and everything other than talent and ability, any metamorphosis is difficult to believe.

Coming back to academics, we had an 8-year-old with CCTGA with intact septae and supratricuspid membrane. So, there was a significant LV inflow obstruction. The membrane needs resection. How to approach the lesion? What are the impacts on the effective physiology? The LV is regressed enough to contraindicate double switch. Is the scenario similar to isolated supramitral membrane? Any experiences?

What is the risk of cath study in an adolescent with suspected Eisenmengarisation? When the baseline saturations are about 90%, with a significant drop on exercise and few ectopics, the obvious risk for any procedure is significant. Are there any guidelines to prove the need and advantages of the cath procedure? Please let me know if anyone has any guidelines on these.

What defines an atrium is probably a perpetual question. If IVC enters an atrium which continues with LV and SVC along with pulmonary veins enter other atrium which continues with RV with a common AV valve in middle, what do you assign it as in an ambiguous situs? We called it {A,D,S}, Levocardia, AV discordance, VA concordance, NRGA. Is there a total correction in such lesions? The caveats are rerouting IVC and pulmonary veins, creating two good AV valves and routing LV to aorta by closing an inlet VSD with subarterial extension. Are the efforts put into such a complex repair worth it? Is it better to resign to a single pump physiology? If anyone had any experience in this regard, please let me know.

When the IVC is interrupted and SVC drains to an atrium to which pulmonary veins also drain, how do you assign the atrial situs? I saw such a baby and had doubts on the technicalities of nomenclature! Any suggestions?

We saw a baby with hemitruncus with clinical signs of operability. The baby is pink, with good pulmonary venous return from both sided veins. The arch shows reversal of flow. However, the PVRI on cath is 23 wood units on left side. The fallacies of PVRI estimate in serial circuit are well known. But, how to be objective in such cases? If anyone knows the way out, please enlighten us.

If a lesion was treated with Glenn circuit few years back and the present evaluation shows possibility of 2-pump repair, can a Glenn be successfully taken down? I understand that such problems are USP of third world, wherein the expertise and decision making were in evolving phases earlier. What are the possibilities for 2-pump in such a scenario? Any inputs?

We are definitely passing through a difficult phase. It is probably the same experience Harry Potter underwent during his first apparition with Dumbledore! Hopefully, the things should resolve. Until, I am not sure of my frequency of posts.

Any suggestions and comments are welcome. Please let me know if you also had any practical difficulties in patient management and of the innovative brainwaves that came to your rescue. 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, of course, only after your permission! (Thanks to Dr Amol Morey for his input sometime back. Although I could not post it for everyone, it was a good learning for us.)

Regards

Kiran