Hello everyone. This is Dr Kiran welcoming you to another post of blog. We shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. This particular anecdote was put up in newspaper article. I happened to call the author and found it to be semiautobiographical. I found this worth sharing. Please go through.

Rakesh was 18 years old. He was never much interested in studies. He always had an ear for music, although he was a lousy singer by himself. He would attend many concerts at his place whenever he found an opportunity, setting everything else aside.

Rakesh’s father owned a small departmental store. They were not super-rich, but were well to do. Father’s ambition was to see a future for his son. As the son was never interested in future studies and had a minimal chances for an office job, his father had intentions of putting him into their business. However, Rakesh never showed any interest in business.

Rakesh had many friends who were equally interested in music. They used to attend the concerts together. Most of them were from middle class families and were not as well to do as Rakesh.

One day the father found Rakesh sitting alone in the living room. “What’s the matter?”, he enquired.

“Nothing great”, replied Rakesh and showed a newspaper item to his dad.

It was about a music concert at a place about 1000 km from their place.

“This man is great. I wish I could have attended his concert”, said Rakesh in a low tone.

His father saw the newspaper item for few moments. “Mind if I join you for the concert?” he asked his son.

Rakesh could not believe his ears. “Really? By all means” he said. “My accommodation problem also solved!” he said silently to himself.

They had a great journey by flight; stayed in a good lodge. They attended the concert. Rakesh was thrilled. His father just sat next to Rakesh throughout the concert.

They were back in the lodge and started packing. They were due for departure early morning.

“I don’t know how to thank you, Dad”, said Rakesh. “I never thought you enjoyed music. All my friends wanted to attend this concert, but you made it for me.”

“Two things” his father said. “First: I don’t enjoy music. But I do enjoy you enjoying it. I wanted to see you happy. So, I joined you all this distance. I am happy that you are happy.”

Rakesh was stunned. His father had driven the point quite well.

“Second”, his father continued. “You said lot of your friends wanted to attend this concert. None could. Largely because they could not afford the travel, stay and all the other expenses.”

Rakesh listened attentively. His father continued.

“In life, both the things should be balanced. One should have a passion: music as in your case. Also, everyone should have a profession which can make the passion possible and reachable. I could make your passion possible because I have a profession which can afford it. Lack of the second would make the first redundant. That is what has happened to your friends.”

Rakesh could appreciate what his father said. His father continued.

“I do not specify the profession. It can be anything of your choice. Government job, private firms, business, contract, whatever it may be. One should have an income enough to sustain the needs of himself and his dependents. At the same time, the needs should not extend the stretchable limits of income. This is the balance one has to achieve. If you wanted to attend a concert somewhere abroad, I probably would not have afforded.”

Rakesh appreciated the genuine tone of his father’s words. He did not feel any preaching. His father sounded more practical than he ever had been.

“In your case, I can give you an option to take care of our store. It is purely your wish. If you want to continue to study, please do so. If you want to find an office job, try it. But, don’t stay stale. Keep doing something constructive or potentially constructive. Early birds usually have an advantage.”

Rakesh wanted to listen, but his dad stopped there. There was some silence. Without many words, they completed packing.

They returned home. That evening Rakesh reached their departmental store. He waited till his dad completed a transaction with a customer. “Yes, Rakesh”, his father asked.

“Dad”, Rakesh replied. “Can you teach me how our store functions? I want to join as a regular salesperson and learn the trade.”

Tears of joy filled the eyes of his father.

If there is anything that we in medical profession miss, then it is the passion for non-medical issues. I have rarely seen medical professionals who have kept the other passions intact. I had heard the story of a famous neurosurgeon who cried on the day of his retirement for not having continued his passion for violin. Also, I knew a medical student who tried to seriously pursue his passion for painting along with medicine and failed in both. It is preferable to have a mature person advising us on how to balance both, but not everyone may find one. This story may tell us something we have already learnt many a times. Yet, it may be worth the pondering.

With this, we shall get back to the interesting learning scenarios.

ALTERNATE PATHWAY

In scientific temper, it is very difficult to accept something without adequately exploring all the possibilities. We had a 3-month-old with Tricuspid atresia 1A. There was only a restrictive PDA. However, the pulmonary venous return was much more impressive than what was suggested by PDA. But on echo, we could not locate any other shunt. A continuous murmur suggested the presence of collaterals, but the same could not be visualised on echo. Since the saturations were less, it was decided to go for a BT shunt. On the table, a coronary fistula was found to distal PA! This explained the picture clearly. Sometimes there is more to the patient than what meets the echo eyes! Please let me know if you have seen similar pictures.

ALL OR NONE?!

We had a 4-year-old with TGA, multiple VSDs, moderate to severe PS with L-malposed great arteries, saturating 85% in room air. On cath, the data appeared to be suitable for ASO with Rastelli repair. The additional VSDs were an issue. The surgical team felt that as many VSDs should be closed as possible. Even then, few VSDs may remain and the final procedure would be a palliative ASO. We were thinking whether such a heroic procedure is worth it. If some VSDs remain, they themselves may be a major cause for concern. Unless we can ensure complete closure of all VSDs, ASO in L-malposed set up may not be worth all the high risk procedure. With the balanced physiology the patient had, one of the contemplations was to leave him alone. What is the opinion of the readership? Let us know your take on this.

RISK Vs RISKIER

We often face problems in those age groups that we are very comfortable with. We had a 21-year-old with cTGA, VSD, PS in the setting of situs inversus. He was saturating 87% in room air. Cath data showed a routable VSD. But his ventricular EDP was 20 mmHg. It was presumed that the raised EDP was secondary to chronic hypoxia and cyanosis. One option was to do a double switch – Senning with Rastelli. The caveat was about the success of Senning in high EDPs. The other option was to leave him as he is now. This may lead to further dysfunction of ventricles with time. Is the option of doing the surgery in situs inversus and its outcome riskier than the medical follow up? What is the natural history of untreated cTGA, VSD, PS? What the average progress of the ventricular dysfunction in such cases? Is there an index for predicting the progress? Such questions remain unanswered. Neither the surgical team nor our team could find an answer for these questions. If anyone has any experiences in these issues, please let us know.

DOUBLE JEOPERDY

One of the inherent weaknesses of the human mind is to fit the findings to a diagnosis that is known! Sometimes, when all the findings are somehow not fitting into a clear picture, we presume that it is a variant of a known diagnosis and try to fit it. Same happened to me one of these days. We had a 9-year-old coming to us with left upper limb hypertension and low BP on right along with absent femorals. I could see a right arch which was narrow at isthmus and the adjacent subclavian showing turbulence at the origin. Since it was a right arch and the echo windows were not to boast of, I presumed a mirror imaging and gave a diagnosis of possible Takayasu disease. However, the cardiac CT undid the actual diagnosis. It was right arch with normal branching, coarctation of aorta at isthmus with aberrant origin of right subclavian artery from the post stenotic segment with the diverticulum of Kommarel. The combination could easily explain the clinical picture. With 2 vessels showing the turbulence with the disparity of pulses, I was lured into making a diagnosis of Takayasu disease neglecting all other markers!! In retrospect, I feel that if I had spent some more time in visualising the arch, I would probably had better chances of making the correct diagnosis. It was a lesson re-learnt. I don’t know how long it will stay! Let me know if you were also lured into making any such diagnosis which caused certain regret on retrospect.

SHUNT IN REGURGITATION

This issue has always troubled me, but did not have a platform to discuss. I am presenting this before everyone for the individual opinion. If anyone happens to find a literature evidence, please support it. The question is: What happens to Qp/Qs in cases of VSD with MR? We had a 6-year-old with complete AV canal defect saturating 92%. He had severe AV valve regurgitation. His calculated Qp/Qs was 1.15:1. Based on the number, he was considered as inoperable. The question was: if there were to be no AVVR, would his Qp/Qs be different? Doesn’t VSD get underestimated in the presence of AVVR? Since AVVR is PVRI independent, the VSD shunt would definitely be influenced by AVVR. Since the AVVR is surgically correctable in this case, the logic of refusing the surgery based on original Qp/Qs correct? The original question still remains. Is the Qp/Qs affected by MR? Or, is the regurgitant volume remains the same and gets nullified in the final equation? Please enlighten.

PEDIATRIC CARDIOLOGY PEARLS:

16. The recommended age for the closure of ASD is around 4 years. This was based on a study in 1983 by Cocherham et al in 87 children with ASD. It is followed as a matter of fact in all places. (Cockerham JT, Martin TC, Gutierrez FR, et al. American Journal of Cardiology 1983 page 1267)

17. An interesting term called double outlet right atrium was introduced by Horiuchi et al in 1976. It involves a primum defect with deviation of interatrial septum to the left. In true sense, it is only AV Canal defect. (Horiuchi T, Saji K, Osuka Y, et al. Journal of Cardiovascular Surgery (Torino) 1976 page 157)

18. Patients with outlet ventricular septal defects usually have deficiency of muscular or fibrous support below the aortic valve with herniation of the right coronary leaflet through the VSD. This leads to progressive aortic valve regurgitation with time. Hence, it is important to recognise this at the earliest and do a surgical correction to avoid any damage to aortic valve. (Van Praagh R, McNamara JJ. American Heart Journal 1968 page 604)

19. The continuous murmur of PDA has the highest number of descriptive names! It was originally described by Gibson in 1900 as having late systolic accentuation and continuation through the second sound into diastole. He, incidentally, never used the term continuous! (Gibson GA. Persistence of the arterial duct and its diagnosis. Edinburgh Medical Journal 1900 page 1)

20. Bland White Garland syndrome refers to ALCAPA. It goes by the names of 3 physicians who described it in 1933. Few have confused it for a bland patient, who looks white because of ischemia and ready for a garland on passing away! (Bland EF, White PD, Garland J. American Heart Journal 1933 page 787)

This brings us to the end of another post. My good friend Dr Prem Alva suggested informing all the followers by email on every update. Sounds practical. If anyone is following the blog and has not become a follower for any reason, please send your email id to me on drkiranvs@gmail.com I shall include your mail id in the list to be informed. Also, send your feedbacks by email or via the comments section.

Regards

KIran