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