Dr Kiran welcomes the readership to the new post. In the last few posts, I tried some innovation in the form of anecdotes which carry greater meanings to life. Thanks for all the positive responses to them. In fact, a couple of inspired friends tried their hands on writing some stuff and with minimal editing from my side, they did a fabulous job. Great going!

From this post on, I am rendering a new idea to the readership. I will be introducing few books in next few posts. These are the books that made a great impact on me in the formative years and still rule lot of my thinking process. I had made some systematic notes on such books all these years. I will be presenting one book per post. We shall start with a small introduction to the author, go on to the works and take one classic of the author. We shall see the essence of the classic and if possible, read some excerpts from the book. If the readership has any different strategy in getting introduced to a book, the advice would be most welcoming.

Let me start with the book I admire. In the year 1998, Dr Howard Cutler, a renowned psychiatrist brought out a book titled “The Art of Happiness: A Handbook for Living”. The twist in the tale was the co-author of the book, who probably did not write a single word for the book! Many thought that the name was a gimmick and Dr Cutler just wanted a bigger publicity for his book. However, Dr Cutler maintained that the contents of book essentially belonged to both the authors. The book became a classic by the might of its contents. The role of the co-author was undeniable, for, he was the Holy Dalai Lama.

The essence of the book is simple; it says “happiness is not a luxury but the purpose of our existence”, taking away the mystical component from happiness. Another shackle breaking thing in the book is the concept that there is a definitive way leading towards happiness! It claims that achieving happiness is scientific and not by chance; it needs discipline to do that.

Some of the points emphasized in the book are:

• A basic way to happiness is to cultivate affection and connection with other human beings.

• The real source of happiness is control of your consciousness, which is impossible without a disciplined mind.

• No matter how powerful they seem, negative emotions and states of mind have no foundation in reality.

• Don’t confuse happiness with pleasure. Pleasure is of the senses and can seem like happiness, but lacks meaning. Happiness, in contrast, rests on meaning and is often felt despite negative external conditions. It is stable and persistent. While pleasures are a bonus in life, happiness is mandatory.

• Happiness is something to be developed over time.

• The fundamental nature of human beings is gentleness; people like to be altruistic if they get a chance.

• Compassion is useful. Rather than being sentimental, it is the basis of communicating well between people. Compassion is not “feeling sorry for someone” but a recognition of commonality.

• Distinguish between love based on attachment and love based on compassion.

The essence of the book is its simplicity. It gives off a sense of the lightness to life, despite all the negative things prevailing in the world. We may not understand everything about our existence; it is all the more important to be good to other beings and to leave the world a slightly better place. It should be our objective and one should not astray from it.

Let me know your comments and criticism on the book and its review. If you have any book which you would recommend to the readership, please let me know. I shall try to get that book introduced.

Let us get back to the real world now: The interesting learning scenarios!

EBSTEIN TENSION

One of the most indecisive lesions for a pediatric cardiologist is Ebsteins anomaly. Every surgeon possibly has a different approach to this lesion. But, has anyone seen an Ebsteins anomaly with severe pulmonary hypertension? Looks paradoxical, but I happened to see a report stating this combination! It was fag end of the day and this 6-day-old presented with this echo report. Unable to believe, I had to re-do the echo. To my surprise, I did find severe PAH, but the Ebstein component was missing! The STL origin was normal, but its tip was tethered to the IVS, causing non-coaptation of TV and the resultant severe TR. The lower half of the IVS was swiss cheesed. How to handle such a scenario? I have started the baby on diuretics, ACEI and asked for a surgical opinion. I shall update this issue when the baby returns for follow up.

LESSER CRIME!

What is the least morbid option in cardiac surgery? The witty answer would be not doing the surgery at all! However, this question came up a serious way in one of our meetings. This 6-year-old girl had VSD with pulmonary atresia with small confluent branch PAs and sizeable collaterals. The options would be BT shunts (possibly bilateral) or unifocalization with/without BT shunt. Considering the complexities and the practical problems in approach, the question was “which is the least morbid?” Jokes apart, it was decided to go for the latter option. I shall update the result after the procedure.

FONTAN FOUNTAIN

I can sense why surgeons are not very keen on Fontan completion. It is often said that the patient should earn his Fontan completion. We had a 4-year-old single ventricle Glenned 3 years back with all parameters favoring Fontan completion. He did quite well in the immediate post-op and was nicely extubated. However, in a matter of few days, he has developed desaturation, respiratory distress and his need for oxygen increased. The Fontan fenestration was seen shunting right to left. Added to it, there was significant pleural effusion. To make the matters worse, a saline contrast injected to a peripheral vein showed contrast in the cardiac chambers, indicating the development of pulmonary AV malformations. The last finding could not be easily explained when there was a flow from IVC to PA, indicated by the gradient across the fenestration. Should such children go for a take-down of Fontan or should they be managed conservatively? Please let me know your opinions on this issue.

BAD COMBO

We had a 3-month-old with DORV and remote muscular VSD. This combination is described in the classification, but is rarely seen. The bad part was the other lesions. This baby had normally related great arteries, severe PAH (No PS), restrictive PDA flowing R to L and coarctation of aorta. Baseline saturation in room air was 80%. Obviously, such combination is not suited for 2-pumpo repair. The coarctation would ensure rapid progress of high PVRI. Since the kid was only 3-month-old, we expected some daring surgeries and eventuality. However, our offer of Coarctation repair with PDA ligation with PA banding plus-minus BTT shunt was not taken by surgeon. The option of PA band does not exist when the baseline saturation is less. The addition of BT shunt would reduce the possibility of future single pump. Moreover, the high risk palliation is not a wise option. With all these explanations, the surgery was deferred. What is your opinion? How would you plan something for this? Let me know your takes on this.

SINGLE OR ONE-AND-HALF?

The pulmonary atresia with intact IVS is a tricky situation. Lot of variables may lead to lot of mistakes in decision making. Predicting their outcome is difficult. We had a 1 year 2 month-old boy with this problem. The TR is severe and the RV is muscle bound. RV apex is not visualized on echo. The pulmonary atresia is membranous. The contemplation of single pump was risked by the TR. One of our upcoming surgeons came up with an idea that is worth sharing. He is considering a Brock’s procedure, passing a cannula across the membranous pulmonary valve, slitting the membrane open using a balloon in the OT. This would be a semi-hybrid procedure! We are indeed excited!! I shall update the outcome in next posts. If anyone has done this before, please let us know.

DARING ATTEMPTS

After a long time, our surgeons have started doing adventures things! We did a Norwood stage 1 and the baby is doing very well. It is very encouraging and we should be doing them more from now on. On the other end, we did a REV procedure in a dTGA, VSD, PS when the VSD was deemed non-routable. Our surgeons enlarged the VSD and did a long LV to Aorta patch followed by a REV. It is very commendable and we all pray for good outcomes in these babies. How many centers are actually attempting REV? If you have any experience with it, please share with us.

PEDIATRIC CARDIOLOGY PEARLS

66. In neonatal IE most cases occur in structurally normal hearts. Although relatively uncommon, increasing numbers of cases of neonatal IE have been reported since the 1970s. This reflects the increased use of prosthetic intravascular devices and more frequent insertion of long-term indwelling central venous catheters (Ferrieri P and others: Unique features of infective endocarditis in childhood. Circulation journal in year 2002 page 2115)

67. The pericardial space, lubricated by lymph, normally contains <30 mL of fluid in the adult and considerably less in infants and children (Holt JP. The normal pericardium. Am J Cardiol in year 1970 page 455)

68. Chorea may be the sole presenting manifestation of lupus, this disease should be considered whenever a child presents with chorea (Arisaka O, Obinata K, Sasaki H, et al. Chorea as an initial manifestation of systemic lupus erythematosus: A case report of a 10 year old girl. Clin Pediatr journal in year 1984 page 298)

69. The seasonal variation of Rheumatic Fever in the temperate climates parallels that of Grp A Streptococcus pharyngitis. Both Grp A Streptococcus pharyngitis and Rheumatic Fever are more common during the winter and spring in temperate climates, but there is no consistent seasonal pattern in the tropics. Geographically, Rheumatic Fever occurs in all latitudes and altitudes (Kumar RK, Rammohan R, Narula J, et al. Epidemiology of streptococcal pharyngitis, rheumatic fever, and rheumatic heart disease. In: Narula J, Virmani R, Reddy KS, et al., eds. Rheumatic Fever. Washington, DC: American Registry of Pathology in year 1999 page 41)

70. In Kawasaki disease, generalized microvasculitis occurs throughout the body in the first 10 days of disease. Myocarditis occurs in the first 3 to 4 weeks, with mononuclear cell infiltration and edema within the myocardium and conduction system. Valvulitis may affect the mitral and aortic valves (Gidding SS, Shulman ST, Ilbawi M, et al. Mucocutaneous lymph node syndrome (Kawasaki disease): Delayed aortic and mitral insufficiency secondary to active valvulitis. J Am Coll Cardiol in year 1986 page 894)


With this, we conclude this post. Please send your feedback to drkiranvs@gmail.com Your suggestions are welcome. Please narrate your experiences with the scenarios discussed and post some novel things seen by you hitherto. Please let me know your opinion on the new section.

Regards

Kiran