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