Dr Kiran hellos everyone. I had a good response for the previous couple of anecdote written by two friends. We shall see another anecdote now, followed by our regular features: Interesting learning scenarios in Pediatric cardiology and the popular “pediatric cardiology pearls”. The anecdote I have written this time was read few years back. I have expanded it a bit, but have retained the first-person narration of the original.

Please read and send your comments:

During my school days, there was a boy who was adored by everyone. He was not the class topper, he was not a champion sportsman, he was not an orator, he did not have great looks, his father was not the richest man in the community, he did not know any music, and he was, certainly, not a celebrity. Yet, he was liked by everyone. For the simple reason that he was good!

I wanted to know what made him so good that he was liked by all. I could sense that I could trust him; I could believe his words for he never lied. I was fond of him because he seemed to have a very simple and honest solution for every problem!

One day, he invited me to his home. I was curious to know where he lived and how his parents were. I went with him.

It was a very humble house; small, neat and clean. The utility of space was evident. I knew that my house was three times as big, but was pretty cluttered.

“Can I meet your parents?” I asked him.

“Both my parents work. They are not back till 6 PM. My elder brother has gone to tuitions and I am alone as of now”, he replied.

I recalled a joke of 10-year-old who answered the door bell of postman with a cigarette in one hand and a beer bottle in the other. “Is your dad in?” asked the postman. The kid laughed and replied, “You are a dumbo. Would you expect me in the house like this if my father was in?” asked the kid! I would have taken the opportunity of solitude to the hilt. Whereas, my friend appeared to be too good for a normal boy!

“You mean, there is no one to watch you over and still you are so good?” I asked him.

“There are certain principles that we live with in my house. They are not rules. There is no punishment if you break them or no reward if you follow them. But, I have experienced that I win applauds by following them. As for getting naughty, yes we do. But it never reaches a stage of destruction!” he replied.

“What rules…er… principles?” I started wondering now.

“Would you like to see them?” he asked doe eyed.

“See the principles?” I wondered.

“Come”, my friend led me towards a wall. There hung a sheet of parchment with neat writing in lines. Almost everyline had a different handwriting.

I read some of them:

 Do unto others as you would have others do unto you
 If you open it, close it.
 If you turn it on, turn it off.
 If you unlock it, lock it up.
 If you break it, admit it.
 If you can't fix it, call someone who can.
 If you borrow it, return it.
 If you value it, take care of it.
 If you make a mess, clean it up.
 If you move it, put it back.
 If it belongs to someone else, get permission to use it.
 If you don't know how to operate it, leave it alone.
 If it's none of your business, don't ask questions.
 If it will brighten someone's day, SAY IT!
 If what you have to say will hurt somebody, DON'T SAY IT!
 If something isn't broken, don't try and fix it.
 If you think you know it all, look around and see how little you really know.

I just kept looking at them. None of those lines seemed great. They were all simple instructions, much like what are written in the operating manuals of appliances. In fact they were nothing compared to some of the “good habits” books I had read. But the simplicity did strike.

“What are these? What exactly these lines have to do with the discipline and good behavior?” I asked him with an element of surprise.

“These are the practical principles that we follow! There is no compulsion of following them. As I told you earlier, there are no rewards or punishments attached to them. But the effect of following them is really rewarding. I do follow them very honestly. You can see what I get from everyone!”

“How do you do it?” was my question, still with the tone of surprise.

“Whenever something good strikes any one of us, we discuss the point during our dinner with all family members. The elders in the family refine the content and make it a single line principle. The person who proposed the principle will get to write the line on the sheet in his own handwriting. Once written, we all try to follow it. As you can see, there are different handwritings on the paper. Every morning, we go to the paper, read everyline with maximum concentration that we can. Even though it is a routine and I know most of them byheart, I try to make this morning affair interesting by analyzing how many of them I have followed the previous day and how many I have broken. When there is a chance for rectifying, I do it honestly. Since they are very simple one liners, we tend to remember them for long time. It has not only won me credits, it has also definitely helped in character building.”

I was struck for a while. I took me sometime to appreciate the simplicity yet the power of routine. “It is so simple to be good, but it is so difficult to be simple” was a bollywood quote I had appreciated sometime back. It seemed to manifest in the simple abode of my friend.

The above anecdote is a true life incident I had read, narrated in first person. “Trifles make perfection and perfection is never trifle” was the life motto of Michelangelo. I felt that not only every house but every workplace should also have such a chart of principles that are practical yet simple. Seeing them everyday would be inspiring and seeing the people follow it may end up whipping the “lazy bums” of the department from their inside. Would you like to have one in your home or workplace?

INTERRUPTED PROBLEMS

We saw a 9-month-old with very complex anatomy. There was a PFO shunting left to right, supramitral membrane, large subaortic VSD, complete (100%) DORV, NRGA, large conus with subaortic obstruction, small bicuspid aortic valve (Z score of minus 3), Type B interrupted aortic arch, severe PAH and large PDA continuing as descending aorta. In a cyanosed crying child, the echo took almost an hour to delineate the anatomy. The mere description of components can discourage the surgeon from any management. How to tackle such cases? It is a combination of Shones complex with complete DORV. The small aortic component does not permit too extravagant surgeries. Is there an option? Can we think of DKS in such cases? Arch reconstruction strategy would depend on what we do proximally. Is there an option of a 2-pump repair in such complex anatomies? Please tell me your opinions on this.

EISENMENGER ISSUE

There was an interesting discussion. Our surgeons maintained that the residual VSD of post-TOF patients, howsoever big the VSD might be, does not develop Eisenmengarization! Physiologically, this statement does not make much sense. But the surgeons maintain their stand, as they have not seen a single case of such physiology till now! Is there any physiological explanation for this? Is it because of the paucity of such cases that it is largely unseen? Let me know your views on it.

TO GLENN OR NOT TO

In a patient of cTGA with VSD with conventional repair (VSD closure to convert it to classical cTGA), is there a role of adding a Glenn shunt if PA pressures are acceptable? One of our senior surgeons felt that a role exists. But, logically, this would offload the MLV. The crux of the issue is MRV which is systemic. The MRV preload and afterload would not change. Why should one add a Glenn shunt in such a scenario? If anyone has any more details on this, please start a discussion.

CUT-OFF LIMIT

Is there a limit for the extent of cyanosis in admixture lesion s as against low Qp? We see huge variations in the levels of cyanosis in low Qp situations. Sometimes, even with good admixture, the SO2 would be low in few. We had a patient of cTGA with DORV and non-routable VSD. The child had SO2 of 65% but the Qp/Qs was 3.8:1! How should such instances be treated? Let me know your takes.

MITRAL PUZZLE

How far the PML mobility characterizes the MV involvement in RHD? We often see children with classical history of acute rheumatic fever with significant mitral regurgitation. Many of them have nicely mobile PML. How should we term them? Should we say that they are a combination of Acute rheumatic fever with non-rheumatic MR? How would the management change? Let me know your opinions.

PEDIATRIC CARDIOLOGY PEARLS

61. In cardiac CT scan, when manually injecting a small dose of contrast, the delay from the start of injection to start of scanning is approximated by a circulation time of approximately 12 to 15 seconds. (Siegel MJ. Multiplanar and three-dimensional multi-detector row CT of thoracic vessels and airways in the pediatric population. Radiology. year 2003 page 641)

62. In the cardiac catheterization, in the absence of a shunt, a step-up of >6% at the atrial level, 4% at the ventricular level, and 4% at the great vessel level will occur in <5% of the time. Variations of >9%, 6%, and 6%, respectively (i.e., three times the standard deviation), would be expected no more than 1% of the time, thus would be highly unlikely in the absence of intracardiac shunting. This is the mark of significant step up in cath data. (Freed MD, Miettinen OS, Nadas AS. Oximetric detection of intracardiac left-to-right shunts. Br Heart J in year 1979 page 690)

63. Shorter refractory periods may facilitate the conduction of very closely coupled impulses and could render the newborn atrium more susceptible to intra-atrial re-entry. This may partly explain the occurrence of atrial arrhythmias such as atrial flutter in the otherwise healthy fetus or newborn infant. (Pickoff AS and others. Atrial vulnerability in the immature hearts. Am J Cardiol in year 1985 page 1402)

64. Even in the current era, congenital heart disease is the most common predisposing cause of brain abscess (Goodkin HP, Harper MB, Pomeroy SL. Intracerebral abscess in children: Historical trends at Children's Hospital Boston. Pediatrics in year 2004 page 1765)

65. Kawasaki disease is a panvasculitis. Most of the morbidity and mortality in affected patients is due to coronary artery aneurysms and associated complications. (Fujiwara H, Hamashima Y. Pathology of the heart in Kawasaki disease. Pediatrics in year 1978 page 100)

That brings us to the end of the present post. Please send your inputs to drkiranvs@gmail.com Since some fresh blood is getting infused to NH team, I hope to give you all something fresh!

Regards

Kiran