Hello everyone, this is Dr Kiran welcoming you to the new post. The objective of the blog is to develop an interest in Pediatric cardiology for curious. We learn few interesting facts and discuss few scenarios on practical issues. As previous, let us start with an anecdote which carries a greater meaning in life. The following anecdote is what everyone goes through in life. Please quote your views on it.

Mr Ray was a worried man. His peace was disturbed due to some problems at his office. He was not willing to discuss it with anyone, for he was afraid people may trifle his problems and start talking of their own. He was of the opinion that no one else could understand the significance, quality or gravity of his problem.

His wife, Mrs Ray could not tolerate the downhill condition of her husband. They had a common cousin who was a motivational speaker and well known “agony-uncle”. She sought his assistance and the man agreed to casually visit the Rays for a non-formal session.

Mr Ray did not know of these arrangements. He welcomed the cousin and both of them started talking. Mrs Ray took this opportunity to re-inform that the cousin was a well known advisor and specialized in solving problems in the work environment.

Mr Ray got interested to know the qualification. He hinted that he had a problem in his office. The cousin acknowledged the hint and told, “Would you like to discuss the problem with me? I can try and help it if you don’t mind.”

Mr Ray took the opportunity. His frustration had been piling up for a while. He thought of shooting it off.

“I don’t know if my problem sounds silly. But, it is killing from inside. I am a very principled man who sticks to discipline. If somebody breaks it, I get very frustrated. More so if I have no control on the person doing it. It multiplies if I get victimized in the process of someone else’s indiscipline”, Mr Ray ranted out.

“You cannot be abstract if you want a solution. Get me the facts straight”, his cousin told with a smiling but straight face.

“I have a colleague”, Mr Ray started. “I don’t want to name him. He was an apprentice when I joined, but he got promoted to the same position as mine in few months of my joining. He did not have the qualification for the post, but the Boss took special interest. Probably because they are from same community.”

The cousin nodded. He knew the rule: Do not break the chain of thought!

“It does not bother me. After all, they manipulated the rules of the firm. In a private firm, the rules are flexible if the Bosses agree. What bothers me is the attitude of the person I do not want to name.”

His cousin interrupted. “Let us call him ‘Nameless’. That will solve the problem of expression.”

“OK”, Mr Ray continued. “Nameless is an opportunistic and lazy person. He quoted his family problems and was scooting off from work. Later, he started quoting the health problems. After sometime, he took off for acquiring new training and qualification. Even now when he has got promoted to a higher position, he continues to do the same. His problems seem to be perpetual and he poses as if no one else in the world has any problems. He wants everyone to share the burden of his problems and there is absolutely no help from his side when the other people have problems”, Mr Ray was increasing his tone of anger and was getting breathless while saying all these.

His cousin was simply nodding. He also took some notes in a piece of paper as Mr Ray spoke.

“The biggest problem is on me. I have to bear all the unfinished work of Nameless. He simply gets up and goes home at his will, well ahead of the closing time of office. I have to stay back extra time to complete his incomplete business. When there is some extra work few days, he simply orders the peon to get all the files and leaves by the time files arrive. All those files get dumped on my table. Of course, the other colleagues also chip in, but I have to take the maximum brunt. I have complained to higher authorities many times, but somehow, they are very sympathetic towards him. They kept telling me that since Nameless does not work full-time, they are cutting a portion of his pay. My earlier Boss who was of the same community as Nameless was so moved by all the pathos uttered by Nameless, that he always gave an out-of-the-turn bonus for him! I recently found out that there was never a pay-cut either for him and my Boss used to present a white lie in front of me to keep me silent. Since then, my anger is burning me from inside.”

His cousin’s face did not show any emotion; neither he gestured any noises of sympathy. He kept jotting up something occasionally.

Mr Ray took a gulp of water. His catharsis was evident. His tone was returning back to normal slowly.

“The problem continues even today. Nameless hardly does any work and gets paid the same amount of salary as I do. Nobody cares on how his work gets transferred to others for completion. For the sake of the office pride, we silently bear the burden and finish the work. My burden is heavier nowadays and I cannot even say a word. I often feel like doing the same thing what Nameless does. I am seriously thinking of going scot-free. I will also do half jobs and put the rest on someone else. Anyway, when my boss is tolerating him, he will have no other option other than tolerating me too.”

Mr Ray took another gulp of water and kept silent.

“Any other people in your office? Any other person with similar nature as Nameless?” his cousin asked Mr Ray.

“There are lots working in my office, but as of now, only Nameless is the exception. Others are hard working. Few, in fact, keep very high standards in work. Few people are really inspirational.”

“Can I tell you a small story?” his cousin asked.

“Sure”, Mr Ray said.

“What I quote are from the Vedas - the divine Hindu scriptures. There is mention of a bird called Vyoma, whose sole aim is to reach the sun. It keeps ascending. It lays its egg en-route its journey. The egg keeps dropping all the way, but since the mother bird was at a very high altitude, the egg has to travel a long distance to reach the earth. Still on its journey down in the sky, the egg hatches and the baby bird is delivered. The baby bird starts of its ascent immediately and starts moving towards the sun. The mother bird continues its journey to sun till it burns off in the way due to the heat. As the Sun is described as a ball of fire and the bird also catches fire and burns out, they become essentially one. Do you find any message in this story?”

Mr Ray was silent. Obviously, it was above his head.

His cousin continued: “The story, as any other story from Vedas, is symbolic of life. It should not be taken in literal sense. The sole pursuit of life is to reach the excellence. It should be so powerful that your progeny picks it up as an inborn, innate nature. No external influence should affect the journey. You should rise so high that what other people talk of you should not even fall into your ears. For, people who stop their journey to talk are simply wasting their life. People with a high objective in life have only one way.”

Mr Ray was not convinced. “Is this advice practical?”

“Of course, yes” his cousin continued. “It depends on where you keep your vision. You can either be the anecdotal Vyoma bird to keep you vision high or you can be a vulture, which keeps flying high but always has its vision fixed on the dead bodies lying on the earth. Here, you are always watching Nameless and getting the vision of a vulture which probably befits the Nameless; not you. Why not keep the vision of some other person in office whom you admire? Why not simulate him? Why not try and get the standards that the other person has achieved? Why not excel and set a standard for others? Why not do the inevitable extra work as a learning experience and gain more out of it? Why should you ever think of going the way Nameless has gone? Would you tolerate yourself if you become what you detest?”

It was hard-hitting for Mr Ray. He was expecting sympathy. What he got was more than what he sought.

“So, how do you think I should rectify the mistakes in the office? Is it not my duty to set it right? Is it not correct to demand my rights for equality and peace of mind?” he asked meekly.

“As a matter of fact, it is not your duty” his cousin said curtly. “You have done your best by bringing it to the notice of your superiors. What they do is not your business. Yes, we all go through phases of injustice in life. It is true that Nameless does not deserve what he is getting. But it happens to almost everyone and almost everywhere. It is neither unique nor rare. If you keep thinking and cribbing about it, you are getting stalled in your journey towards excellence. You are wasting your time by underachieving your potential. You are a creative man. You can be of greater pertinence to the world. Only that you have to think in the correct direction.”

Mr Ray was speechless. He took sometime to digest what he heard. Slowly, he got up from his chair, shook hands with his cousin and said, “I may not agree with all that you said, but seems to make sense in a way. I shall try to implement and let you know the results”.

This anecdote is symbolic of what happens in everyone’s life. In every working place, a certain percentage of people are found who live a parasitic life on others. We used to call it 80:20 principle in the government run hospital I worked earlier. Overall, 20% of honest staff did 80% of all the work, but those 80% staff was struggling hard to get all the credit for the good work not done by them. The scenario may not be much different in any private set up either. There are always people who make a living by sycophancy; they just need a gullible boss! There are people who think being smart is better than being honest. There are people who allow their conscience to collect their full salary without working for it. There are people who are first to attend the meetings with higher authorities and take lead in presenting data for which they have not even contributed. There are people who believe in getting their work done by others by any hook or crook. Such people may present as witty, jovial, smart, wise-cracking and popular with everyone except for their colleagues who actually end up doing the pending work of such characters. But all those apparently smart qualities are largely to hide their lazy self. It is a frustrating experience to be colleagues of such kind. The above anecdote shares one way of dealing with such people. Such breed is so common that each one of us can recall such a character in our working place. How are your experiences in dealing with such people? How do you make yourself compatible in such situations? Please share your experiences with other readers. It is really good to have some solace from every possible corner!

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

SMALL INTO LARGE: POSSIBLE?

We had one year old girl with Tricuspid atresia IIB with disproportionate branch PAs. The LPA was far smaller than the RPA. This baby also had bilateral SVCs with LSVC far bigger than the RSVC. Cath data showed suitability for Glenn shunt. Now the problem was attaching the big LSVC to small LPA! We have seen BTT shunts that cannot exceed the size of the native ipsilateral PA. Is the rule applicable to Glenn too? Is it possible to attach a 10mm SVC to a 5mm PA? How are the dynamics different? The surgical team had a split opinion on this. Please let me know your take on it.

IMPROVEMENT IS PROGRESSIVE AND VICE-VERSA!

This one was learning experience. We had a two-year-old single ventricle- DILV. Cath data for Glenn suitability showed a mean PA pressure of 18mmHg. It was above the comfort level of our surgical team. We were about to negate the possibility of surgery when one of our senior surgeons (who is otherwise very conservative) opted to do the Glenn in this baby. It was a matter of surprise and we wanted to know the basis for his opinion. He explained in very simple terms that Glenn will take off about 30% of his systemic venous input into the heart and about 15% of total cardiac output in this baby. So, post-op, the PA pressures are likely to come down by about 15%, bringing it into comfort zone! It was an eye-opener for mathematically challenged people like some of us! But the logic appeared very appealing. The offloading concept of ventricles is the basis for Glenn shunt. When the criteria are made, is this mathematical calculation already incorporated? That is, do we fix a mean PA standard after making correction for the post-op reduction or not? I could not find an answer. If anyone knows this, please let me know.

LOCATION LOGISTICS

I had brought up this question earlier. In Ebstein’s anomaly if there is a communication between LV and atrialised RV, can we call it a VSD?! One more facet came up this time. It was a 6-year-old girl with Ebstein’s anomaly with the communication between LV and atrialised RV low down and had an aneurysm of STL projecting up and partially covering the defect! Can we call this “restriction of VSD”? Here, neither the term VSD nor the direction of STL limiting the flow is semantically confirmed! Can STL send the aneurysm towards the direction of RA and be still called by the same name? Let me know your ideas on it.

HOW EARLY IS EARLY?

It is tough to picture the cause/effect relationship in retrospect. We had a 2-day-old newborn with Tetralogy – absent pulmonic valve complex. It came with respiratory distress. On echo, the branch PAs were dilated. The chest radiograph showed pneumothorax on the right. The condition deteriorated in a couple of days and the neonate was intubated and electively ventilated. The repeat chest radiograph showed a pneumonic patch with no evidence of pneumothorax this time. The question is: Can absent pulmonic valve complex produce respiratory symptoms so early? Is it beneficial to get a cardiopulmonary CT scan? What is your experience of early presentations in such entities? Please let me know your take on this.

CAUSE – EFFECT

We had a 7-month-old with heart pushed to right. Echo revealed TOF with small RPA. The return from right sided pulmonary veins was minimal. Chest radiograph showed a totally collapsed right lung. CT chest done outside was reported as hypoplastic left lung. Is the lung status acquired or congenital? Is the small RPA the cause of right lung hypoplasia or vice-versa? Is there any way of determining the cause-effect relationship? Please let me know your opinion on this issue.

PEDIATRIC CARDIOLOGY PEARLS:

41. Reporting echocardiography in a systematic manner conveys meaning for management. As per the norm, right-sided and left-sided structures at each level are evaluated according to their morphology, their relative positions, their connections to proximal and distal segments, and the presence and location of shunts, obstructions, and valvular regurgitation. (Edwards WD. Congenital heart disease. In: Schoen FJ, ed. Interventional and Surgical Cardiovascular Pathology: Clinical Correlations and Basic Principles. Philadelphia: WB Saunders, 1989:281-367)

42. The only prospective control trial of Carvedilol use in children with heart failure was published by Shaddy et al in 2007. The trial showed an improvement in children less than 24 months on echo parameters. However, on composite end points of study, there was no significant benefit. (JAMA, 2007, page 1171)

43. Shepard et al in 1991 reported that 1 in 40 patients with tuberous sclerosis may die as a direct result of cardiac rhabdomyomas. (Mayo Clin Proc 1991;66:792-796)

44. When cardiac myxomas obstruct the semilunar valves, patients experience symptoms while bending forward or lying down, with relief of symptoms when standing. (Robertson R. Primary cardiac tumours: Surgical treatment. Am J Surg 1957;94:183-193)

45. Rheumatic mitral stenosis severe enough to result in symptomatic heart failure may occur in the first two decades of life in developing countries. (Agarwal BL. Juvenile mitral stenosis in developing countries: Problems and challenge. J Assoc Physicians India 1986;34:141-144)
With that, we come to the conclusion of one more post. I am really interested to know if there are any readers of this blog other than me! Please send your inputs via the comments section or to my email id drkiranvs@gmail.com The purpose of this blog is to make it interactive. Hope someday it happens!

Regards

Kiran