This is Dr Kiran welcoming everyone to the new post of the blog. As we have seen, the primary objective of this blog is dissemination of genuine interest in Pediatric cardiology to all those who have got smitten by this charming bug! I had the opportunity of writing a lot of historical details about the subject and drugs used in this field. For the past few weeks, I have been writing a few short anecdotes which may touch the readers somewhere and may bring back some memories. None of these stories are my own. Most of them are heard, read or sent by the friends. It may seem personal if there is lot of relevance, but that is purely coincidental! With this disclaimer, I am moving on to the following anecdote which was sent by a friend. He did not specify if it is his own. But, the quality of story was worth sharing. Please go through:

Ram was 14 years old. His adolescence was more visible on his recent “don’t care” attitude. He was fond of science classes in the school and was quite good at it. He would often tease his father with the question, “Dad, what is relativity?” His accountant father was probably unaware of who Einstein was! Father used to just smile and keep quiet.

A new circus company had campaigned in their town. Ram wanted to see the circus. It was almost the month-end and his dad did not have spare cash for the new expense. Somehow, after taking a hand-lone from a colleague in the office, the father-son duo went for the show.

They were standing in the queue. A parallel lane was buying tickets from another counter. There was one group in the parallel lane. It was a big group with eight children, all under the age of 12. Ram felt that they didn't have much money. Their clothes were frayed but clean, and the children were well-behaved all of them standing in line, two-by-two holding hands in back of the man who had brought the kids. They were jabbering about the clowns, elephants and other acts they would see that evening. Ram, who had gone to a circus a couple of years back, could sense those kids had never been to the circus before. That evening looked like a highlight of those young lives.

Ram and his dad were behind a couple of people in the line when the children’s group reached the counter. “One full and eight half tickets, please” the man said. The lady in the counter told the amount. The man opened few notes of currency from his pocket and counted. His face fell sad. He counted the money again. “How much did you say the amount was?” he asked the counter lady again. He was turning pale with the answer.

Ram and his father were witnessing the scene. Suddenly, Ram’s dad pulled up a note of Rs 100, dropped it on ground and told the man in the opposite lane: “Sir, you have dropped this note. It fell from your pocket.”

The man looked at Ram’s father. His eyes were filling with tears. He meekly accepted the note and bought the tickets. He came towards Ram, held the hands of his father and told in a choked voice, “I don’t know how to thank you. These are the children from the neighboring orphanage. What the donors pay is just enough for food and clothes. They were dreaming day and night about the circus. As their caretaker, I could not resist bringing them here. I got some of my money and asked for some from my friends. Still, I fell short of some. Bless you, Sir; can’t repay your kindness.”

When Ram reached the counter, they did not have enough money for the tickets. They just walked away from the counter. After few yards, Ram’s dad held hands of his son. “Can we just postpone our programme by a week?” he asked Ram.

“No problems, dad”, Ram said. “Three things, actually. First, I can wait for a week. Second, I can do without too; I have seen the circus earlier. Lastly, today you have taught me what relativity is!” Ram said with a glee in his face.

Ram had found a new meaning for life that day. Moreover, he decided never to tease his father again.

The above anecdote is quite touching, especially for people who empathise. The community of Doctors probably has highest chances for empathy. One of the doctor friends, who does community service told me about her experience with patients wherein she had to make decision for them imagining herself in their shoes! It is a tough experience. The relativity of the issue probably depends on how many tough situation we have passed through in our life. There is the story of a man who was cursing God for not having footwear till he saw a man without legs! The cursing suddenly got transformed to praise. The quantum paradigm shift in this instance is evident. Life is relative and the meek ones need to know this well before they decide on something drastic. Understanding who is right is probably not as important as what is right. This is the biggest aspect of effective leadership, management, parenting, teaching and coaching. It's amazing how much we can accomplish if we simply focus on leaving everyone we meet in better shape than we found them.
With this, let us get back to our regular feature: Interesting learning scenarios

RIGHT PARACHUTE

We can all recall a number of parachute mitral valves. When the papillary muscle of left ventricle is alone, the entire chordae end up getting inserted to the same site and the physical picture is of a parachute with the pointing end at pap muscle and the balloon at the annulus. This is possible for the mitral valve where the site of insertion is well defined. How about the tricuspid valve which sends its chordate over a wider area? We saw a 9-month-old with all the tricuspid chordae getting attached to a single pap muscle. On searching the literature, we found the existence of such an entity. Anderson et al had reported this in a setting of TGA and Aziz et al for TOF. In our case, it was a large VSD and a small ASD. If anyone has any data on this entity, please let us know.

ATRIAL OUTLETS

Another interesting variant of tricuspid valve was seen by us. We have seen a double orifice mitral valve. However, a double orifice tricuspid valve is a rare variant. We had such a scenario in a 6-month-old wherein the TV had two openings on either side of interventricular septum. This was associated with a large inlet VSD and a small RV. The entire MV and one opening of TV were to the MLV and the other opening of TV was to the small MRV. It was an eventual single pump repair. The literature shows the existence of this condition without any other heart lesions. If you can recall any instance of noticing such an anatomical variant, please put up your experience.

PARTIALLY TRANSITIONAL

What would you term a Transitional AV canal defect in which the VSD has closed by a septal pouch? What is the terminology to be used? It is taught that the inlet VSD does not close. However, we have often seen a small inlet VSD of transitional AV canal defect closing spontaneously over a period of time and becoming a partial AV canal defect. Should we continue to call it transitional or change to partial? Tell me your views.

RIMS AND LOCATION

Of late, we have been largely successful in eliminating the need of Transesophageal echocardiography for ASD device closures. Our transthoracic echos have been found sufficient for ASD devices. In this process, we often find that the IVC rims being unsteady. Many times, the defect is not visible at all when the IVC is opened in the subcostal saggital view or in the short axis view. In such cases, we have found a mixed success rate. Is there is fool-proof method in the TTE for this problem? How many centres have actually dropped the TEE for ASD device closures? What are the experiences in those places? Please let us know.

SIDE TO SIDE!

Continuing the problems with devices, we have seen few muscular VSDs which look perfectly OK for device closures during echo evaluation. However, on the table, the LV side of the defect would be much larger than the RV side. Not only crossing becomes a problem, the size of the device to be used is also an issue. Since the muscular VSD devices are symmetrical, the LV side of the device may be too small for the defect and the RV side may be correspondingly big. Our experience with the VSD devices is not as extensive as our PDA and ASD devices. How are the other centres managing this issue? Please let us know if there is a way.

PEDIATRIC CARDIOLOGY PEARLS:

46. Sildenafil seems to be beneficial in the management of Primary pulmonary hypertension. Acute vasoreactivity studies in PAH patients suggest that sildenafil may have greater acute hemodynamic effects than inhaled nitric oxide and may further reduce pulmonary vascular resistance. (Michelakis E, Tymchak W, Lien D, et al. Oral sildenafil compared with inhaled nitric oxide in PAH. Circulation. year 2002 page 2398)

47. AHA guidelines on IE prophylaxis extend to HCM. Bacterial endocarditis appears to be virtually confined to patients with the obstructive form of HCM, with a prevalence of <1%. Vegetations most commonly involve the anterior mitral leaflet or septal endocardium at the site of mitral valve - septal contact and less commonly the aortic valve. ( Spirito P, Rapezzi C, Bellone P, et al. Infective endocarditis in hypertrophic cardiomyopathy. A good review is done by authors. Circulation. year 1999 Page 2132)

48. Levosimendan is a calcium-sensitizing agent that has been evaluated in adults with acute decompensated heart failure and in patients with chronic heart failure. One pharmacokinetic study has been performed in children with congenital heart disease. There is no increase in myocardial oxygen consumption or arrhythmias. (Turanlahti M, Boldt T, Palkama T, et al. levosimendan in pediatric patients evaluated for cardiac surgery. Pediatr Crit Care Med. year 2004 page 457)

49. In cases of myocarditis with congestive heart failure, digitalis may be used and has effected dramatic improvement in many instances. However, during periods of acute inflammation, the myocardium may be hypersensitive to digitalis, so rapid administration to achieve therapeutic levels should be avoided. (Parrillo JE. Myocarditis: Good review article on treatment. J Heart Lung Transplant. year 1998 page 941)

50. In Pediatric restrictive cardiomyopathy, beta-blocker therapy was suggested to blunt rapid heart rates in their patient population in whom significant ST-segment depression was noted at higher heart rates. However, tolerating the therapy is the chief caveat. (Rivenes SM, Kearney DL, Smith EO, et al. Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy. Circulation. year 2000 page 876)

With that, we come to the end of another post. Please send your views, opinions, criticisms either by the comments box or by email to drkiranvs@gmail.com I shall post them on your behalf. I am preparing the list of books about which I can talk in next few posts. Till then, I shall continue with the anecdotes. If you have any interesting short stories to share, please mail them to me. Your contribution would be acknowledged.

Regards

Kiran