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

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