This is Dr Kiran welcoming everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for all enthusiastics. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. This small story got reprinted in a prominent daily. It brought out my old memories of fascination when I had read it few years back. Just see how you feel about it!

A couple of decades back in a small town lived a man famous for his intelligence. He was well read and respected. People used to come to him for discussing their problems. He would give them the advice, free of cost. In a way, he was popular as a wise man.

For obvious reasons, certain people in the town disliked him. They wanted to demean him. Since they would not be any match for him on a direct confrontation, they were seeking surrogate opportunities.

One day, someone told the man, “The local politician has been spreading the word that your son is an idiot. Do something”.

The man was surprised. His son was in 4th standard and far from idiocy! He was a clever boy, even though he never topped the class.

The man thought of getting the facts straight. He called his son to chamber and asked, “Do you know the local politician?”

“Yes”, the son answered with a twinkle in his eyes.

“He was saying something bad about you. Any tussle between you?”

“Oh, ignore him, Dad. He is not a smart man”

The man was surprised. The comment was too much for a boy of nine.

“You must not use such terms for elders. I have come to know that the man thinks you are an idiot. But I know that you are not. What makes him think so?”

The boy grinned. “The politician fellow has no good business. He sits with few of his friends everyday in the porch in front of his house. I have to go the same way to reach the school. On seeing, he calls me near him, keeps a 5 rupee coin in one palm and one rupee coin in the other. He asks me to pick up one coin. Once I pick up the coin, he laughs loudly and tells his friends: “Did I not tell you?” asks me to keep the coin for myself. Later all of them laugh again. This happens every day.”

The man was interested. “And what coin do you pick up?”

“Obviously, the one rupee coin”

The man was surprised. “I think you know that 5 is more than one?”

“Of course Dad, I know”

“Still you pick up the one rupee coin?”

The boy looked at his dad in surprise. He excused himself and left the room. He came back within a minute with a glass jar in his hand. The jar was three-fourth full of one-rupee coins.

“What is this?” the man demanded.

“This is to show who the idiot is!” the son laughed.

The man was still unsure whether he understood the situation correctly. “Explain” he told his son.

“It is wits versus stupidity. The moment I pick up the five-rupee coin, this joke would end. The politician has no other business than proving me wrong. I oblige. This will continue as long as I keep picking up the single rupee coin. I want to keep the game alive. Now, I want you to tell me whether 5 rupee coin is worth more or this jar.”

The man was taken aback. His son looked the smartest kid on the world to him at that moment.

The mere feeling that we are better or smarter than others sounds clichéd. The joke is usually on us when we deliberately seek to outsmart others. I recently came across a man who had manipulated the system so effectively that he has his cake and has been eating it too. Only because he makes the people around him feel superior. Every place has such smart people. We often find juniors outsmarting the egoistic seniors, class IV outsmarting their bosses and so on. Whether the act is cheating or outsmarting or manipulating depends on where we stand. If our perspective is of the boy in the story, then we are outsmarting. If it is of the politician, we are getting manipulated! The hunter and the hunted need not be permanent. The places can change. The anecdote is only serves a reminder for better introspection before we attempt to demean the others. Never sure who the victim is!

With this, we shall get back to the regular feature: Interesting clinical scenarios.

SMALL Vs BIG RV

We came across a toddler with TOF physiology with small Tricuspid valve annulus. The resultant RV was suboptimal in size. The decision was for a one-and-a-half ventricle correction. Since pulmonary annulus was small, the decision was to get a transannular patch done. How does such a physiology affect RV and Glenn shunt? Can the free PR be detrimental for RV function? Can the TAP take away the purpose of Glenn shunt in reducing RV preload? Even if RV sustains the load, can the increased preload of RV increase the eventual PA pressures and cause Glenn shunt to fail? If anyone has seen this combo working, please let us know the expected dynamics of post op status.

MASS(IVE) DECISION

A 10-year-old boy was admitted to our ICU with rapidly deteriorating LV function. He came in clear LVF. After the basic resuscitation, we did his echo. The LA and LV were dilated and LV EF was about 20%. Behind the LA, a dense, homogenous mass was visualised. The mass was compressing on the LA, deforming its contours. Moderate MR was seen. One of our colleagues noted a thickened mitral valve with restricted mobility of posterior leaflet. Some pericardial effusion added to confusion. Since the mass did not appear to have much effect on the ventricles and the history was acute, he suspected the diagnosis to be rheumatic, with associated mass. To add on, ASLO titres were positive, ESR and CRP were high. There was no clinical history suggestive of rheumatic fever. Still, the patient was started on steroids in suspicion of indolent rheumatic carditis in failure suggestive of rheumatic activity, along with supportive measures and antibiotics. The patient made a very good recovery on this, with EF increasing to 50% in 48 hours of therapy. We got a CT scan, which showed a homogenous mass behind the heart. The Hounsfield unit measurements favoured the diagnosis of lipoma. With the improvement of EF, repeat echo showed a normal motion of mitral valve, taking away the suspicion of RHD! We have stopped steroids and observing the child, with rest of the supportive treatments on. There are some pertinent questions to be answered. How did a slowly growing lipoma produce the mass effect all of a sudden? If no other super-added disease process is adding on, how do we explain the rapid deterioration in this boy? What post-operative histology can explain all the sequence of events? We are yet to get the surgery done for him due to infection. Once we get further answers, I shall brief you. But the rarity of the case is interesting. Lipomas in the mediastinum are not very frequent. That too, massive ones are very rare. We got just one case report in the literature. If any new waves of thought occur, please post them.

IMMUNE DECISION

How good are the screening tests for primary immune deficiency in a country like India? With such a massive population and rampant consanguinity, our numbers in primary immune deficiency must be enormous. However, no medical student with substantial number of years in paediatrics can recall many such children. Comparatively, our database on HIV is better, thanks to awareness and availability of diagnostic modalities. We had a 9-month-old with vegetations on both AV valves and aortic valve. The child had a significant history of abscess formation with IM injections. Our primary suspicion was immune deficiency. But, primary screening for that turned out to be negative. HIV screening in child and both parents was negative. We involved our hemato-oncologist into the picture, but the scenario did not become any clearer. Assays of immuneglobulins are just one part of the diagnosis. How about the complement function, leukocyte functional assay, receptor level abnormalities and others? Our fellows vehemently chased the diagnosis, but we are financially stressed. We did not want to burden the parents over massive expenditure on investigations. Has anyone come across cases like this anytime? How did you go about the diagnosis? Also of interest is the methodology to be followed in India if a suspected case of primary immune deficiency comes when the immune globulins are of normal levels. Please let us know your viewpoints on it.

UNGUARDED EBSTEINS

What is the DD for Ebsteins anomaly!? The question sounds a bit tough, as there can hardly be any. The picture of Ebsteins is so clear that even partially trained fetal echocardiographers vouch by the diagnosis. We had a baby with such an eventuality. It was reported as Ebsteins from outside. What we found surprised us. It was a case of unguarded tricuspid valve! The leaflets were in the right place with no displacement. But, none of them coapted with each other, leaving an unguarded opening to allow a free to-and-fro movement of the blood. The RA was enlarged and the moderator band looked thick and mobile, giving an impression of displaced STL. Is this condition common? It is possible to have missed this condition for Ebstiens. Please let me know if you have seen such entity earlier.

AGELESS REGURGITATION!

How would the pulmonary regurgitation behave with age in setting of absent pulmonic valve? We had a 24-year-old with TOF and absent pulmonic valve! His PR was minimal, but the PAs were huge. The latter findings suggest that the PR must have been there and significant. How to explain the minimal PR that we find now? No one could recall any patient of this age with such findings. Does the free/severe PR improve with age in absent pulmonic valve syndrome? If anyone has come across such a scenario, please let us know.

PEDIATRIC CARDIOLOGY PEARLS:

26. There is a sub-variant of Partial AV canal defect called Transitional type. In the transitional form of partial AVSD, there is aneurysmal replacement of a portion of the inlet ventricular septum, leaving a minimal VSD with a tricuspid pouch which usually obstructs any major shunting at the ventricular level. (Seward JB, Tajik AJ, Edwards WD, et al. Congenital heart disease. In: Two-Dimensional Echocardiographic Atlas. Vol. 1. New York: Springer-Verlag, 1987)

27. In 1973, when echocardiographic evaluation was not a part of diagnosis, it was suggested that the diameter of the ventricular septal defect compared to the diameter of the ascending aorta could be used in determining the prognosis. When the ratio was ≥ 0.8, this defect was considered large, was unlikely to spontaneously close, and the infant would have a large pulmonary blood flow and pulmonary artery hypertension. Although the determinants are changed with the advents of Echocardiography, few still consider this point as a significant one. (Rowe RD. In: Barratt- Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy. Diagnosis and Surgical Treatment. Proceedings of the Second International Symposium. Edinburgh: Churchill Livingstone, 1973 page 121)

28. In Atrial Septal defect, Eisenmenger reaction was found to be 7% in the first decade, 8% in the second decade, 10% in the third decade, and 11% in the fourth decade and beyond. (Cherian G, Uthaman CB, Durairaj M et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. American Heart Journal 1983 page 954)

29. The commonest congenital heart disease with right aortic arch is common arterial trunk (popularly called Truncus arteriosus) (Anderson RH, Thiene G. European Journal of Cardiothorac Surgery 1989 page 481)

30. In d-TGA newborns, increased numbers and size of pancreatic islet cells, and increased weight of the adrenal cortex are found. These findings in the pancreas and adrenal cortex are similar to those seen in infants of diabetic mothers and support the contention that the higher-than-usual glucose concentration in the descending aorta during fetal development may play a role (Naeye RL. Archives of Pathology 1966 page 412)

This brings us to the end of one more post. I had sent the emails to all the known followers last time. 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