Hello everyone, Dr Kiran’s welcome to all for the new post. The objective of the blog is to develop a dissemination of Pediatric cardiology for everyone interested. We learn few interesting facts and discuss few scenarios on practical issues.

Before we start our anecdote, let me congratulate our Fellowship students: Dr Sripadh Upadhya and Dr Vishal Changela, who finally had their exams last week! They have performed very well and are sure to pass out with flying colours. Dr Changela is a very honest and sincere worker. He has amazing patience while he teaches someone and often called “teacher material”! Dr Upadhya is likely to join the NH team. Good luck for the future of both these fellows.

Also, let me take this opportunity to welcome Dr Dhanya as our new fellowship student under RGUHS. Good luck for her stay and career.

With that, let us start with an anecdote as we have been doing in past few posts. The present anecdote was sent to me by mail by a friend. It is told in many management classes and motivation seminars. Enjoy:

An offbeat lecturer who was known for his eccentric ideas came to the class. He was supposed to take the class for the final year degree students. He carried a couple of bags with him.

He wished the class and opened his bags. The students were interested on what he would do without starting the regular class.

He got a glass jar out and kept it on the table. He started pouring glass marbles into it till the brim.

“Is this glass jar full?” he asked the class. “Yes, sir” came the reply.

“Is it?” the lecturer said. He carefully placed few more marbles on the pile till it started falling.

“Now, is it full?” he questioned again.

“Yes, sir” was the reply, more emphatic this time.

“Let us see” the lecturer said and brought out some fine pieces of stone and started putting into the glass jar. The stone pieces percolated down and settled in the spaces between the marbles.

“Now, what is your opinion?” he asked.

“The jar now seems to be full, sir” came the reply.

“Let us see”, the lecturer said. He took his bag and took out some fine sand. He poured the sand carefully over the jar. The sand occupied the spaces between the stone. The lecturer looked at the class.

The class went silent. Nobody wanted to commit.

“I know what you are thinking. The jar now is full. Is there anything I can fit in?” the lecturer pulled out a bottle of water and started pouring it into the jar, which seamlessly got accommodated!

“I have just demonstrated how the perception changes in life”, the lecturer began. “Our perceptions of ‘full’ and ‘fill’ are very relative. First time, adding one more marble would have dropped it out. But we had forgotten the spaces in between. So, ‘full’ was by the perception of marbles, not for space in the jar.”

The class was listening.

“Our life may seem quite filling with the job we do” lecturer continued, “but there is always scope for few more things. It need not be as major as our primary profession. It can be an activity, a hobby, a service for someone or anything. If the jar in this example was your life, the marbles would be your profession. As you saw, the jar was full with marbles, but was still empty. Life would not be a fulfilling experience unless you fill it with something more than your profession.”

The silence of the class was broken by a loud applause.

The above anecdote is self explanatory. I have seen people who keep complaining about lack of time for pursuing anything other than their job. However, they would nicely discuss a recent cricket match, a new movie, some odd soap series in the television and so on. Ask them about their reading habit, the answer would be a sharp ‘where is the time?’ Not that they do not find time, but they do not recognize it. Probably, reading does not feature in their list of priorities. I know how hard my present boss works. He is in the hospital by the time we reach and keeps going on even when we leave. I find it surprising how he manages time to read a lot. He seems to have read quite lot of contemporary literature. When I was in a book festival, I called him to ask if he wanted any book. He could easily tell me 3-4 books which he liked to read. Some of them were part of a series of which he had the initial volumes and had read them already. It is really commendable and not many actually know of this quality of his. It may be prudent to take a cue from this anecdote and try something new, which would be a fulfilling experience in life.

With this, let us get back to our regular feature, interesting learning scenarios:

OPERABILITY REGURGITATES!!

In a given scenario of operability decisions in echo for cases with severe PAH, the chamber dilatation and pulmonary venous return play the vital roles. For example, in a case of large VSD with bidirectional shunt on echo, we look at pulmonary venous return and LA/LV enlargement to ascertain the operability. We happened to see a child recently which put us into confusion. The child had multiple muscular VSDs which shunted bidirectional. There was severe TR with flow reversal in IVC. Hence, there was RA/RV dilatation. As the TR was severe, the forward flow across the pulmonary valve was probably compromised. This may result in low pulmonary venous return. That compromises both components of operability in echo. Is there any study on how a severe TR compromise Qp in cases VSD? Is the hypothesis of low pulmonary venous return in severe TR correct? Please let me know your take on it.

ONE MORE; ONE LESS

How frequent are the combination lesions with TAPVC? As a feature, we have seen TAPVC being a part of heterotaxy isomerisms. But when the TAPVC is the primary lesion, presence of defects other than a mandatory ASD are not very frequent. I have initially mentioned of a TAPVC with intact IAS, but a large VSD. We found TAPVC with severe coarctation of aorta recently. It was difficult to diagnose to begin with, as the flow across aortic valve itself is less in TAPVC. The likelihood of underestimating coarctation is very high. However, this child had a very clear 2-D picture and gradients diagnostic of coarctation. The combination of high Qp with obstruction to Qs is not very common. The same was confirmed in the operating room. Please let us know your experiences of such atypical combinations.

DUAL DIFFICULTIES

One more variant of TAPVC was seen on the same day as the previous scenario. A 6-month-old came with features of increased Qp. We found a dilated coronary sinus and the common confluence of pulmonary veins entering the CS. We prepared the report of cardiac TAPVC. But, the routine suprasternal view picked up something more. We could see another channel with the colour Doppler showing the flow towards the probe. We started tracing the origin of flow and it went into the common confluence. We reconfirmed the presence of dilated CS and the entry of common confluence into it. It was an instance with the common confluence of pulmonary veins communicating with more than one exit channel. In our case, it was communicating with CS and via ascending vertical vein into the SVC and RA. The combination is very well described, but not frequently seen. It reconfirmed the sayings that our seniors repeatedly told us: Always see all the possible pathways of exit in a TAPVC. If our complacence permitted us to stop at the level of CS, we would have lost an important finding of surgical implication. Let us know your experiences of TAPVCs with dual drainages.

WHY SHOULD NOT WE?!

Several logical explanations are put up on why a small PDA be closed. The risk of endocarditis and the ease of device closure top the list. In the same logic, why should we spare a small muscular VSD? The VSDs described as “maladie de Roger” are the small mid muscular VSDs with high gradient, often with a thrill but no volume overload of left heart. Such lesions have risk of endocarditis, but are too much for open heart surgical risk. Extending the logic of small PDAs, the risk can be eliminated with the device closure of such VSDs. With the learning curve for muscular VSD device closures getting sustained in many centers, why should our guidelines be changed for the favor of device closure of such lesions? Give your opinion on this issue.

THE QUEST FOR CORONARIES

One of the popular viva questions in pediatric cardiology involves coronaries. “Where all in pediatric cardiology do you need to chase the coronaries?” Usual expected answers are TGA, TOF, Kawasaki, ALCAPA, VSD with intact IVS, coronary fistulae, coronary collaterals and ruptured sinus of Valsalva. We had a situation in which none of the above featured and yet, our chase for coronaries was essential! It was a 7-month-old child weighing 3 kg with aortic atresia, VSD, normal sized ventricles, large AP window feeding the ascending aorta, type B arch interruption and the PDA continuing into descending aorta! “Why not a truncus” was the logical question. The answer was in coronaries. We could not see proximal aorta at all. The coronaries were visualized, but their origen was elusive. We could not connect the origin of coronaries with the single great vessel that arose from RV. This finding was against the diagnosis of trunk, which by definition should give rise to systemic, pulmonary and coronary circulations. We have asked for a CT scan, but are not really sure if CT can pick up the origin of coronary. The child looked operable on echo. But the plan may be tedious. Translocating the coronaries, routing LV to common vessel, closing the AP window on the pulmonary side, creating RV to PA homograft, correction of type B interruption and PDA closure!! The procedure may take an entire day in the OR for the braveheart surgeon. We are awaiting the cardiac CT report. I shall keep you informed on the progress. Please let us know if any such diagnostic puzzles were encountered involving the coronaries in your experience.

PEDIATRIC CARDIOLOGY PEARLS

36. In pulmonic stenosis, the pulmonary ejection click corresponds to the time when the doming pulmonary valve reaches its open position. The more severe the stenosis, the earlier in systole the click occurs, until it merges with the first heart sound and becomes inaudible. The intensity of the click varies with respiration, decreasing during inspiration and increasing during expiration. These findings enable a confident physical diagnosis. (Vogelpoel L, Schrire V. Circulation journal 1960 page 55)

37. In VSD with Pulmonary atresia, the pulmonary circulation is dependent on PDA or aortopulmonary collaterals. Ductal and collateral sources may coexist in the same patient but only rarely coexist in the same lung. (Liao PK, Edwards WD, Julsrud PR, et al. Journal of American College of Cardiology 1985 page1343)

38. The risk of tetralogy of Fallot increases in the siblings of an affected child. Nora and Nora have suggested sibling recurrence risk of 2.5% if one sibling was affected, and 8% if two or more siblings were affected (Nora JJ, Nora AH. American Journal of Medical Genetics 1988 page 137)

39. In truncus arteriosus, the truncal valve was tricuspid in 277 (69%), quadricuspid in 86 (22%), bicuspid in 35 (9%), pentacuspid in 1 (0.3%), and unicommissural in 1 (0.3%). (Fuglestad S, Puga F, Danielson G. American Journal of Cardiovascular Pathology 1988 page 39)

40. The original classification of Tricuspid Atresia by Kuhne in 1906 was impractical, yet survived as not many were bothered! Once the treatment modalities started, there was a need to refurbish the classification. The present modification of the classification of Tricuspid Atresia as types 1,2 and 3 and subtypes A, B and C was given by Rajesh Tandon and JE Edwards in 1974. (Tandon R, Edwards JE. Journal of Thoracic and Cardiovascular Surgery 1974 page 530)

This brings us to the end of one more post. I am planning to introduce few books of interest for the medical personnel outside their subject. Let me know your take on this plan. Use the comment box for your ideas and suggestions. Use my email id if you find any problem in using the comments box. 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.

Regards

Kiran

Dr Kiran welcomes everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for everyone with a taste for this phenomenal subject. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. As a part of my interest in medical history, I came across this extraordinary piece of courage and conviction which I felt like sharing. Please go through this and discuss.

Tylenol is the brand name for paracetamol, which is a household name in USA. It is one of the most popular over-the-counter medications and a household name. It was manufactured and marketed by Johnson and Johnson. In contrast to the tablets from of present day, it was available in capsule form 3 decades back.

In 1982, something terrible happened. Eight people who used the Tylenol capsules died. A psychopath claimed that he had put cyanide in some of the capsules. No further information was available as which batches were affected and how many such capsules were likely contaminated.

If you are the boss of a company who has something like 31 million bottles of the drug in the market at that moment with few possible contaminated capsules (claimed and not proven), how would you react?

Random sample testing? Financial assistance to the deceased? Legal battle on proving the non-involvement of the company? Animal testing? Methods to educate the public on ways to find out the non-contamination? Denial? Prove that the deaths are not related to Tylenol? Bribe the officials to get a clean chit? Bury the issue? Some more skeletons in the cabinet? What else?

Mr James Burke, the CEO of J&J at that time was a smart man and a smarter businessman. He could have done anything mentioned above or even more. But what he “did” was amazing.

He opted for full cooperation with the media immediately. He personally appeared on one TV program after another to take responsibility and keep people up to date on the situation. He ordered withdrawal of all the 31 million bottles of Tylenol from the market! He offered to replace the possibly contaminated capsules by Tablets, whose packing was fool-proof. On behalf of the company he accepted responsibility and made it sure to everyone that J&J had not actually done anything wrong, but still is taking the measures for damage control.

It cost J&J a whooping $100 million! More for the replacement in the form of tablets. For the mistake not committed by them.

What J&J gained out of this could not be measured by money. Its way of handling the situation and the decisions taken won applauds from media. "What Johnson & Johnson executives have done is communicate the message that the company is candid, contrite and compassionate, committed to solving the murders and protecting the public," noted the Washington Post.

But what J&J had lost was not just money. It had to recreate the trust. It is not easy when a big thing falls. And Tylenol was the biggest J&J had. It invested heavily in the restoration job. Its share was estimated to be around 37% in the market."It will take time, it will take money, and it will be very difficult; but we consider it a moral imperative, as well as good business, to restore Tylenol to its preeminent position," said James Burke.

It was the responsible handling of the situation that probably saved the day for J&J. It was too big a catastrophe for easy recovery. J&J made it only because its CEO stuck to the ethics, forgetting the money. The Tylenol crisis brought values of J&J into sharp relief. Instead of bringing in a contingency plan, the company carried on by expressing the principles and values. The public could see the transparency of effort, magnitude of the exercise and more importantly, a principled leadership.

Very shortly, public had placed Tylenol on its top position. Its market value enhanced. People could see why they need to trust the brand. Moreover, they were not ready to disown a company which had social responsibility of the magnitude they displayed.

Today, the market share of Tylenol is much larger than what it used to be. Just to end the anecdote, it did not take J&J more than 2 years to recover their financial losses. What they gained out of the display of honesty was priceless.

It takes good principles and strong base of ethics to be a leader. There are bosses so thimble that they cannot even reprimand an undisciplined subordinate! If a problem is brought to their notice, the first thing they do is to demoralize the whistle-blower! They find the whistle-blowers to be a big nuisance. Good or bad, the show must go on with minimal tension for them. Internal nexus with people of cheap morals for ulterior motives by others in the company does not bother them. Honesty need not be honoured; indiscipline need not be punished; cheating someone of their rights need not be corrected – all for own peace of mind. The definition of success in the present day corporate culture depends on how many controversies the leader avoids. After all, avoiding resolving a problem or denying the existence of a problem is much simpler than solving it. Why put your hand into a troublesome beehive when the the affected person is not you?!

Contrasting such scenarios of the present day is what makes James Burke a magnanimous character. He accepted the problem instead of brushing it aside despite his company did not really have anything to do with the actions of a psychopath; the entire thing had happened outside the production and despatch. He instilled a discipline amongst his staff by setting up a crisis management team. He identified the key people who needed to be involved, and limited the number of spokespersons. Most important of all, he took the lead and worked hard in executing the morals. The whistle blowers were honoured. No lazy bum was spared. Profits and losses did not matter in front of ethics. He did not let any internal nexus or outsider encash the situation to their profit. The cash flow would have suffered as the brand was the biggest they had. He accepted it. He sought a lion’s share of trouble by being in the air and media, taking questions, justifying the stand of his company to public and so on. If we can name any one person with maximum loss of sleep and peace in this fiasco, it was the leader, James Burke.

It is just to see what prevails in the end. The loose characters in corporate history who “ruled” with a single objective have perished shortly. It is the people with courage and ethics whose names are remembered today. It is for anyone to choose what they want to be.

Type Tylenol and James Burke in Google. Read the amazing story with many other details when you are free.

With this, let us get back to our regular feature: Interesting leaning scenarios:

DOUBLE LEFT: OK; DOUBLE RIGHT: TOO!

We happened to see a child with a double orifice tricuspid valve. I do not remember to have seen this before. This particular child had one orifice communicating with RV and the other with LV! A large VSD was associated. An ASD had decompressed the RA, so the flow gradient was not found across the TV orifices. Overall, the hemodynamic effects of the lesion were effaced due to coexisting lesions. If such a lesion is seen by you earlier, please let us know how it was and how it behaved.


TOO EARLY OR TOO LATE?

We often come across such situations. We had a 7-month-old first timer who presented with a large PDA. The operability was unclear in the clinical evaluation and echo. On cath, the pulmonary and systemic pressures were identical and Qp/Qs was 0.9:1. Calculated PVRI was 7.3. Is the condition due to failure of primary pressures to fall or rapid development of PVRI? Both possibilities will have opposite ends of management. Should we do something or leave? Would a trial of sildenafil help? Someone suggested a partial ligation of PDA. It will control the Qp in former case if the primary pressures drop. Otherwise, it will act as a pop-off and help the overall survival. It sounded practical, but is it accepted? Please let me know your experiences of handling such situations.

UNACCOUNTABLE

Few children come with diastolic challenges. The picture remains unclear even with the last investigation we can think of. One such had come to us one year back with left ventricular failure. She had a subaortic membrane with moderate gradient across LVOT. Medical management did not get along. She was operated on and the membrane was resected. With a ferocious post-operative battle by team of our intensivists, she could be discharged. She did not come back for follow up for one year and recently, returned with biventricular failure. There was no subaortic membrane this time, but the LVOT gradient was severe. Being refractive to medical therapy, we catheterized her to get a better delineation of anatomy. The EDPs were very high (30-45 mmHg). Her LVOT gradient was 70 mmHg. We projected a high risk and asked surgical team to take a call. They wanted to go in and take the risk of “act of commission”. However, she could not take make it to OT; died a day prior to proposed surgery. The scenario was depressing. We wanted to get a pathological and histochemical diagnosis, but the family was not for it. If such scenarios are seen earlier, please enlighten us on the possible lines of management.

DEVIL AND DEEP BLUE SEA

It is difficult to take a stand in some of the situations. We had a 22-year-old with TOF and diminutive RPA. About 9 years back, he had undergone a BTT shunt. He came back now with history of exercise intolerance and increasing cyanosis. We could not see the BTT shunt on echo. We expected the shunt to have got blocked. On cath, we found a different scene. The RPA had grown a little to about 6 mm. The LPA was big. The left lung had received a good quantity of blood from BTT shunt for the past 9 years and had become hypertensive. The PVRI of left lung was very high. This had lead to the tardiness of BTT shunt. Management was the issue now. Doing nothing Vs doing another palliation. Since the patient was symptomatic, some felt that another shunt should be created to RPA. However, the risk of another procedure, high risk palliation, proposed benefit and other factors took precedence and the patient was decided on medical management. What is the opinion of readership? How would you go about in such cases?

COLOUR FACTOR

An interesting discussion happened between two of the senior consultants of our team recently. The cardiologist quoted a “big man from the west” of having avoided transannular patching in Tetralogies. The statistics and the outcome presented in the data were impressive. However, our surgeon was not impressed. He quoted the anatomical variations of the Hispanic populations versus oriental populations. He said that the Asian anatomy demands transannular patching where as the western may not. These statements open up the possibility for epidemiological research. If anyone knows any further data on this, please let us know.

PEDIATRIC CARDIOLOGY PEARLS

31. In CCTGA, Complete heart block may be as high as 10% at initial presentation and the incidence is cumulative as the age progresses (Bharati S, McCue CM, Tingelstad JB, et al. American Journal of Cardiology 1978 page147)

32. Although the incidence of congenital bicuspid aortic valve is as high as 1.3 to 2% of general population, only 2% of patients with congenitally abnormal aortic valve will experience significant stenosis or regurgitation by adolescence (Bonowro, Carabello B, de Leon AC Jr, et al Journal of American College of Cardiology 1998 page148)

33. Although anterior leaflet of the mitral valve appears to be bigger, it is not so. The posterior mitral leaflet is longer at its base and shorter in its basal-to-apical length than the anterior leaflet. Hence, both the leaflets have approximately the same area (Ranganathan M, Lam JHC, Wigle ED, et al. Circulation journal 1970 page 459)

34. In the mitral valve, congenital cleft is directed anteriorly toward the outflow septum or aortic root in contrast to the cleft in atrioventricular septal defect, which is directed towards the interventricular septum (Smallhorn J, de Leval M, Stark J, et al. British Heart Journal 1982 page 109)

35. A correlation has been found between the anatomy of mitral valve and the central nervous system, more in the behavioural aspects. The subendocardial surface on the atrial aspect of the middle portion of the mitral valve is rich in nerve endings, including afferent nerves; mechanical stimuli from this area caused by abnormal mitral valve coaptation may cause abnormal autonomic nerve feedback between the central nervous system and mitral valve nervous system. Floppy Mitral Valve innervation patterns with distinct nerve terminals provide a neural basis for brain - heart interactions, augmented by mechanical stimuli from the prolapsing Floppy Mitral Valve (Boudoulas H, Schaal SF, Wooley CF. Floppy mitral valve/mitral valve prolapse: Cardiac arrhythmias. In: Vardas PE, ed. Cardiac Arrhythmias, Pacing, and Electrophysiology. London: Kluwer Academic Publishers, 1998: page 95)

This brings us to the end of one more post. I have been answering any comments in the same comment box. So, please go through the comment box if you have posted any idea. I have been sending emails to all the known followers since last couple of times informing about the update. Since we do not have a visitor counter, I will not know of your visit if you do not comment. Use the comment box for your ideas and suggestions. Use my email id if you find any problem in using the comments box. 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.

Regards

Kiran

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

Welcome to the readership. This is Dr Kiran inviting you to another post of blog. As previous, we shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, as we have been doing since past posts, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. The following story was told by one of the motivational speakers in a seminar. The speaker was author of multiple best-sellers and does motivational courses nowadays. Please go through this interesting anecdote.

A mother came with her adolescent son to a wise man. It was clear that the boy was brought there by emotional force! He looked uninterested.

“Sir”, the mother addressed the wise man. “This is my son. He is very intelligent, but not focused in life. I request you to give some advice to him”.

The wise man smiled at the boy, who frowned impatiently.

The wise man was indeed wise. He was a modern day Guru. He would not preach or advice atonement or punishments. His words were always more practical than many would think.

“Can we talk about something?” the wise man invited the boy into conversation.

The boy thought of leaving. He looked out of the window. A freshly asphalted road was seen. A small ball of tar was lying at a corner.

“Let us talk about that small ball of tar” the boy said, trying to ridicule the wise man.

The wise man did not get offended. “Can you please get that ball of tar?” he asked.

The boy felt happy. He wanted the “wise man” to give back nicely. He fetched the tar ball and gave it.

“How much do you think this tar ball would cost?” the wise man asked the boy.

“Cost? It is useless; worthless” the boy replied.

“These are basic hydrocarbaons, aren’t they? How much do you think its raw material would have costed?” the wise man had a conviction in his words this time.

“Boy, this fellow knows some chemistry too” the boy thought. He wanted to show off now. “May be, about ten bucks” he answered cautiously.

“Hmmm. Let us get few steps back. This is made of the same basic chemicals used to make plastics I believe?”

“Yes”, the boy was enthusiastic this time. “Vinyls and other polymers would have the basic molecular skeleton as this”

“High quality polymers are expensive. Is the same basic chemical composition applied there also?”

“That is correct. The hydrocarbons have same basic chemical structure. They can be sent through different pathways to obtain different substances. But the basic chemical skeleton remains the same”, the boy was feeling proud this time.

“I hear some of the finest and highest quality materials used in rockets and medical instruments can be prepared using the same chemical framework. I was told by a scientist that these substances cost almost Rs 10,000/- per meter” the wise man said with an innocent tone in his voice.

“You have heard him right. These are very high quality substances that need to be segregated at a very early stage of hydrocarbon processing. The channelization actually matters. Of course, high level of discipline and proper instrumentation along with appropriate environmental standards make all the difference”, the boy knew his stuff and was eager to show off.

“I was told mere ten grams of the basic substance can make high quality thread as long as 1,000 meters. Can it?”

“Of course. Such material used in critical processes may cost about Rs 1,000 a meter” the boy said with full vigor in his voice.

“So, you mean to say this useless, worthless ball of tar could have been something worth a million rupees only if were to get channelized at the beginning”, the voice of wise man was impassionate.

Silence. The boy did not say anything.

“Don’t you think the same process gets reflected in life?” the wise man saw into the eyes of the boy.

The boy bent his head. Words were struck in his throat. “Yes” he said slowly.

“Power, knowledge and intelligence may not reach where it deserves unless it gets a sense of direction and channelized into the proper pathway. It is left to you whether you wish to end up Rs1000 a meter or a waste mass of material at a deserted corner of road”, the wise man stopped.

The boy choked. The message was conveyed.

I remember one of my physics lecturers telling the class. “Be a vector. Your energies are nothing without the element of direction.” Not many of us could understand or appreciate what he told at that age. However, the words remained with me. I see a greater meaning now for the same. Every day, the same message gets extrapolated in many fronts. Even when it comes to management of heart problems, we often see how the small steps kept at early phases of disease process end up being great benefits for the child. Similarly, how small mistakes at the inception end up being a disaster for the patient. Appropriate channelization, whether in life or in any trifle looking setting, is indeed a big task. I felt like sharing this anecdote for the final punch it delivers and the resultant impact!

With this, it is time to get back to our regular feature: Interesting case scenarios:

INEVITABLE LAST MINUTE

We had 8-year-old with single ventricle physiology with IVC interruption and pulmonary atresia and intact IAS. How would the cath for single ventricle suitability help? We cannot enter the PA, cannot get the pulmonary venous wedge or have any way of measuring the PA pressures. Since it is a potential Kawashima, unless the data is clear, the decisions are pending. We contemplated this, yet went on with cath. Cath study did not spring any surprises and no clear data could be obtained. It was left to “on the table” decision making. Is there any other way of getting the data from cath? Please let me know your opinions on such scenarios.

POSSIBLY POSSIBLE!!

Is there a possibility of transposition of great arteries with {S,D,S} combination? In other words, can we have normally related great arteries in transposition diagnosis? The words sound paradoxical. However, we came across such a report done from outside. On repeating the echo, we found a DORV, NRGA, VSD, severe PAH. The commitment of PA was unclear and could be routed to RV or LV depending on how the patch is to be directed. When we put up the question on the possibility of outside report, we were surprised to hear that {S,D,S} in TGA is possible and reported. Has anyone come across this combination? If so, please send the details. We shall put it up in the subsequent posts.

CALCIUM DYNAMICS: IS PREDICTION POSSIBLE?

We had one 11-year-old girl, who had undergone RV to PA homograft conduit repair about 11 months back. The girl came back with calcification of homograft with severe obstruction. On the cath study, we found calcification on the VSD patch also! The surprise element was the rapidity with which the calcification had occurred and progressed. The surgical team introspected about the type of valve. They wanted to see the records to find out if it was an antibiotic prepared valve or a cryo preserved valve. Does it actually matter? Is there any way in which we can predict the calcification? Are there any observations regarding these issues? Please let me know your experiences on this.

NUMERICAL DILEMMAS

Sometimes, the initial assessment of a patient with high Qp and sever PAH shows clear operability on clinical assessment and basic investigations. However, the cath data on the same patient may not show the same picture. We had one 8-year-old of CCTGA, VSD with PAH. He was saturating 98%. However, the surgical team sought a cath study to ensure the anatomy. On the cath, the PVRI was 14.8 wood units! The surgery was ruled out due to the numbers. Many of us would have come across such situations. Is there a cut off for ordering additional investigations? If there is a disparity between the numerical values and clinical findings, doesn’t the latter get precedence? How to resolve such issues? If you have found any solution for these scenarios, please let us know.

COLLATERAL DAMAGE

We come across scenarios wherein the patients with single ventricle physiologies saturating well would be put on Glenn shunt for reducing the volume loads. We had a 2-year-old with single pump physiology saturating 92% in room air. He had good aortopulmonary collaterals. How should we go about dealing with these collaterals after the Glenn shunt? Do the saturations come down if we ligate the collaterals? Is a low SO2 after surgery acceptable? If we leave the collaterals, is the risk of overflow and volume load higher? The senior surgeons always maintain that the collaterals are self limiting and if jobless, they would involute by themselves. Is that true? If so, how long do they take? Is accepting low saturations better than risking overflow and volume load? The surgeons were reluctant due to issues related to approach. They suggested that we can coil the collaterals post op. What is more logical? If any other institute has any consensus on this issue, we would be interested to know their logic.

PEDIATRIC CARDIOLOGY PEARLS:

21. Arterial malformations have two vascular patterns: Arterio Venous Malformations (AVM) and Arterio Venous Fistulae (AVF). AVM (microfistulas) are multiple arterial feeders joined via a nidus to draining veins. AVF (macrofistulas) are direct shunts between large arterial and venous channels. (Avery JB, ed. Cardiovascular Pathology in Infants and Children. Philadelphia: WB Saunders, 1984 page277)

22. The relationship between development of aortic arch and migration of neural crest cells into the pharyngeal arches was given for first time in 1991 in avian embryo studies. (Kuratani S, Kirby ML. American Journal of Anatomy 1991 page 215)

23. Normally, there are two right and two left pulmonary veins. The most common variation is the presence of a single pulmonary vein on either the right or left side, with a prevalence of about 24% in anatomic studies. Contrary to the popular belief, the prevalence of a third pulmonary vein on either the right or left side is only 1.6% to 2%. (Healy JE Jr. Journal of Thoracic and Cardiovascular Surgery 1952 page 433)

24. The persistent Left SVC opens into coronary sinus and drains into Right atrium. Hence, the coronary sinus enlarges in presence of left SVC. This interferes with blood flow from the left atrium into the left ventricle. An increase in the magnitude of the left-to-right shunt at the atrial level was found in patients with secundum atrial septal defects (ASDs), persistent LSVC, and dilated coronary sinus. (Byerregaard P, Laursen HB. Acta Paediatrica Scandinevia 1980 page 105)

25. Left axis deviation in ECG is found in moderate VSD. However, large VSDs and equal ventricular pressures demonstrate right ventricular hypertrophy pattern in ECG. (van den Heuvel F, Timmers T, Hess J. British Heart Journal 1995 page 49)

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