Dr Kiran welcomes everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for everyone with a taste for this phenomenal subject. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. As a part of my interest in medical history, I came across this extraordinary piece of courage and conviction which I felt like sharing. Please go through this and discuss.
Tylenol is the brand name for paracetamol, which is a household name in USA. It is one of the most popular over-the-counter medications and a household name. It was manufactured and marketed by Johnson and Johnson. In contrast to the tablets from of present day, it was available in capsule form 3 decades back.
In 1982, something terrible happened. Eight people who used the Tylenol capsules died. A psychopath claimed that he had put cyanide in some of the capsules. No further information was available as which batches were affected and how many such capsules were likely contaminated.
If you are the boss of a company who has something like 31 million bottles of the drug in the market at that moment with few possible contaminated capsules (claimed and not proven), how would you react?
Random sample testing? Financial assistance to the deceased? Legal battle on proving the non-involvement of the company? Animal testing? Methods to educate the public on ways to find out the non-contamination? Denial? Prove that the deaths are not related to Tylenol? Bribe the officials to get a clean chit? Bury the issue? Some more skeletons in the cabinet? What else?
Mr James Burke, the CEO of J&J at that time was a smart man and a smarter businessman. He could have done anything mentioned above or even more. But what he “did” was amazing.
He opted for full cooperation with the media immediately. He personally appeared on one TV program after another to take responsibility and keep people up to date on the situation. He ordered withdrawal of all the 31 million bottles of Tylenol from the market! He offered to replace the possibly contaminated capsules by Tablets, whose packing was fool-proof. On behalf of the company he accepted responsibility and made it sure to everyone that J&J had not actually done anything wrong, but still is taking the measures for damage control.
It cost J&J a whooping $100 million! More for the replacement in the form of tablets. For the mistake not committed by them.
What J&J gained out of this could not be measured by money. Its way of handling the situation and the decisions taken won applauds from media. "What Johnson & Johnson executives have done is communicate the message that the company is candid, contrite and compassionate, committed to solving the murders and protecting the public," noted the Washington Post.
But what J&J had lost was not just money. It had to recreate the trust. It is not easy when a big thing falls. And Tylenol was the biggest J&J had. It invested heavily in the restoration job. Its share was estimated to be around 37% in the market."It will take time, it will take money, and it will be very difficult; but we consider it a moral imperative, as well as good business, to restore Tylenol to its preeminent position," said James Burke.
It was the responsible handling of the situation that probably saved the day for J&J. It was too big a catastrophe for easy recovery. J&J made it only because its CEO stuck to the ethics, forgetting the money. The Tylenol crisis brought values of J&J into sharp relief. Instead of bringing in a contingency plan, the company carried on by expressing the principles and values. The public could see the transparency of effort, magnitude of the exercise and more importantly, a principled leadership.
Very shortly, public had placed Tylenol on its top position. Its market value enhanced. People could see why they need to trust the brand. Moreover, they were not ready to disown a company which had social responsibility of the magnitude they displayed.
Today, the market share of Tylenol is much larger than what it used to be. Just to end the anecdote, it did not take J&J more than 2 years to recover their financial losses. What they gained out of the display of honesty was priceless.
It takes good principles and strong base of ethics to be a leader. There are bosses so thimble that they cannot even reprimand an undisciplined subordinate! If a problem is brought to their notice, the first thing they do is to demoralize the whistle-blower! They find the whistle-blowers to be a big nuisance. Good or bad, the show must go on with minimal tension for them. Internal nexus with people of cheap morals for ulterior motives by others in the company does not bother them. Honesty need not be honoured; indiscipline need not be punished; cheating someone of their rights need not be corrected – all for own peace of mind. The definition of success in the present day corporate culture depends on how many controversies the leader avoids. After all, avoiding resolving a problem or denying the existence of a problem is much simpler than solving it. Why put your hand into a troublesome beehive when the the affected person is not you?!
Contrasting such scenarios of the present day is what makes James Burke a magnanimous character. He accepted the problem instead of brushing it aside despite his company did not really have anything to do with the actions of a psychopath; the entire thing had happened outside the production and despatch. He instilled a discipline amongst his staff by setting up a crisis management team. He identified the key people who needed to be involved, and limited the number of spokespersons. Most important of all, he took the lead and worked hard in executing the morals. The whistle blowers were honoured. No lazy bum was spared. Profits and losses did not matter in front of ethics. He did not let any internal nexus or outsider encash the situation to their profit. The cash flow would have suffered as the brand was the biggest they had. He accepted it. He sought a lion’s share of trouble by being in the air and media, taking questions, justifying the stand of his company to public and so on. If we can name any one person with maximum loss of sleep and peace in this fiasco, it was the leader, James Burke.
It is just to see what prevails in the end. The loose characters in corporate history who “ruled” with a single objective have perished shortly. It is the people with courage and ethics whose names are remembered today. It is for anyone to choose what they want to be.
Type Tylenol and James Burke in Google. Read the amazing story with many other details when you are free.
With this, let us get back to our regular feature: Interesting leaning scenarios:
DOUBLE LEFT: OK; DOUBLE RIGHT: TOO!
We happened to see a child with a double orifice tricuspid valve. I do not remember to have seen this before. This particular child had one orifice communicating with RV and the other with LV! A large VSD was associated. An ASD had decompressed the RA, so the flow gradient was not found across the TV orifices. Overall, the hemodynamic effects of the lesion were effaced due to coexisting lesions. If such a lesion is seen by you earlier, please let us know how it was and how it behaved.
TOO EARLY OR TOO LATE?
We often come across such situations. We had a 7-month-old first timer who presented with a large PDA. The operability was unclear in the clinical evaluation and echo. On cath, the pulmonary and systemic pressures were identical and Qp/Qs was 0.9:1. Calculated PVRI was 7.3. Is the condition due to failure of primary pressures to fall or rapid development of PVRI? Both possibilities will have opposite ends of management. Should we do something or leave? Would a trial of sildenafil help? Someone suggested a partial ligation of PDA. It will control the Qp in former case if the primary pressures drop. Otherwise, it will act as a pop-off and help the overall survival. It sounded practical, but is it accepted? Please let me know your experiences of handling such situations.
UNACCOUNTABLE
Few children come with diastolic challenges. The picture remains unclear even with the last investigation we can think of. One such had come to us one year back with left ventricular failure. She had a subaortic membrane with moderate gradient across LVOT. Medical management did not get along. She was operated on and the membrane was resected. With a ferocious post-operative battle by team of our intensivists, she could be discharged. She did not come back for follow up for one year and recently, returned with biventricular failure. There was no subaortic membrane this time, but the LVOT gradient was severe. Being refractive to medical therapy, we catheterized her to get a better delineation of anatomy. The EDPs were very high (30-45 mmHg). Her LVOT gradient was 70 mmHg. We projected a high risk and asked surgical team to take a call. They wanted to go in and take the risk of “act of commission”. However, she could not take make it to OT; died a day prior to proposed surgery. The scenario was depressing. We wanted to get a pathological and histochemical diagnosis, but the family was not for it. If such scenarios are seen earlier, please enlighten us on the possible lines of management.
DEVIL AND DEEP BLUE SEA
It is difficult to take a stand in some of the situations. We had a 22-year-old with TOF and diminutive RPA. About 9 years back, he had undergone a BTT shunt. He came back now with history of exercise intolerance and increasing cyanosis. We could not see the BTT shunt on echo. We expected the shunt to have got blocked. On cath, we found a different scene. The RPA had grown a little to about 6 mm. The LPA was big. The left lung had received a good quantity of blood from BTT shunt for the past 9 years and had become hypertensive. The PVRI of left lung was very high. This had lead to the tardiness of BTT shunt. Management was the issue now. Doing nothing Vs doing another palliation. Since the patient was symptomatic, some felt that another shunt should be created to RPA. However, the risk of another procedure, high risk palliation, proposed benefit and other factors took precedence and the patient was decided on medical management. What is the opinion of readership? How would you go about in such cases?
COLOUR FACTOR
An interesting discussion happened between two of the senior consultants of our team recently. The cardiologist quoted a “big man from the west” of having avoided transannular patching in Tetralogies. The statistics and the outcome presented in the data were impressive. However, our surgeon was not impressed. He quoted the anatomical variations of the Hispanic populations versus oriental populations. He said that the Asian anatomy demands transannular patching where as the western may not. These statements open up the possibility for epidemiological research. If anyone knows any further data on this, please let us know.
PEDIATRIC CARDIOLOGY PEARLS
31. In CCTGA, Complete heart block may be as high as 10% at initial presentation and the incidence is cumulative as the age progresses (Bharati S, McCue CM, Tingelstad JB, et al. American Journal of Cardiology 1978 page147)
32. Although the incidence of congenital bicuspid aortic valve is as high as 1.3 to 2% of general population, only 2% of patients with congenitally abnormal aortic valve will experience significant stenosis or regurgitation by adolescence (Bonowro, Carabello B, de Leon AC Jr, et al Journal of American College of Cardiology 1998 page148)
33. Although anterior leaflet of the mitral valve appears to be bigger, it is not so. The posterior mitral leaflet is longer at its base and shorter in its basal-to-apical length than the anterior leaflet. Hence, both the leaflets have approximately the same area (Ranganathan M, Lam JHC, Wigle ED, et al. Circulation journal 1970 page 459)
34. In the mitral valve, congenital cleft is directed anteriorly toward the outflow septum or aortic root in contrast to the cleft in atrioventricular septal defect, which is directed towards the interventricular septum (Smallhorn J, de Leval M, Stark J, et al. British Heart Journal 1982 page 109)
35. A correlation has been found between the anatomy of mitral valve and the central nervous system, more in the behavioural aspects. The subendocardial surface on the atrial aspect of the middle portion of the mitral valve is rich in nerve endings, including afferent nerves; mechanical stimuli from this area caused by abnormal mitral valve coaptation may cause abnormal autonomic nerve feedback between the central nervous system and mitral valve nervous system. Floppy Mitral Valve innervation patterns with distinct nerve terminals provide a neural basis for brain - heart interactions, augmented by mechanical stimuli from the prolapsing Floppy Mitral Valve (Boudoulas H, Schaal SF, Wooley CF. Floppy mitral valve/mitral valve prolapse: Cardiac arrhythmias. In: Vardas PE, ed. Cardiac Arrhythmias, Pacing, and Electrophysiology. London: Kluwer Academic Publishers, 1998: page 95)
This brings us to the end of one more post. I have been answering any comments in the same comment box. So, please go through the comment box if you have posted any idea. I have been sending emails to all the known followers since last couple of times informing about the update. Since we do not have a visitor counter, I will not know of your visit if you do not comment. 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
ಭಾನುವಾರ, ಜೂನ್ 20, 2010
ಶನಿವಾರ, ಜೂನ್ 12, 2010
This is Dr Kiran welcoming everyone to the new post. The objective of the blog is to develop a meaning dissemination of Pediatric cardiology for all enthusiastics. We learn few interesting facts and discuss few scenarios on practical issues. Before that, let me start with an anecdote. This small story got reprinted in a prominent daily. It brought out my old memories of fascination when I had read it few years back. Just see how you feel about it!
A couple of decades back in a small town lived a man famous for his intelligence. He was well read and respected. People used to come to him for discussing their problems. He would give them the advice, free of cost. In a way, he was popular as a wise man.
For obvious reasons, certain people in the town disliked him. They wanted to demean him. Since they would not be any match for him on a direct confrontation, they were seeking surrogate opportunities.
One day, someone told the man, “The local politician has been spreading the word that your son is an idiot. Do something”.
The man was surprised. His son was in 4th standard and far from idiocy! He was a clever boy, even though he never topped the class.
The man thought of getting the facts straight. He called his son to chamber and asked, “Do you know the local politician?”
“Yes”, the son answered with a twinkle in his eyes.
“He was saying something bad about you. Any tussle between you?”
“Oh, ignore him, Dad. He is not a smart man”
The man was surprised. The comment was too much for a boy of nine.
“You must not use such terms for elders. I have come to know that the man thinks you are an idiot. But I know that you are not. What makes him think so?”
The boy grinned. “The politician fellow has no good business. He sits with few of his friends everyday in the porch in front of his house. I have to go the same way to reach the school. On seeing, he calls me near him, keeps a 5 rupee coin in one palm and one rupee coin in the other. He asks me to pick up one coin. Once I pick up the coin, he laughs loudly and tells his friends: “Did I not tell you?” asks me to keep the coin for myself. Later all of them laugh again. This happens every day.”
The man was interested. “And what coin do you pick up?”
“Obviously, the one rupee coin”
The man was surprised. “I think you know that 5 is more than one?”
“Of course Dad, I know”
“Still you pick up the one rupee coin?”
The boy looked at his dad in surprise. He excused himself and left the room. He came back within a minute with a glass jar in his hand. The jar was three-fourth full of one-rupee coins.
“What is this?” the man demanded.
“This is to show who the idiot is!” the son laughed.
The man was still unsure whether he understood the situation correctly. “Explain” he told his son.
“It is wits versus stupidity. The moment I pick up the five-rupee coin, this joke would end. The politician has no other business than proving me wrong. I oblige. This will continue as long as I keep picking up the single rupee coin. I want to keep the game alive. Now, I want you to tell me whether 5 rupee coin is worth more or this jar.”
The man was taken aback. His son looked the smartest kid on the world to him at that moment.
The mere feeling that we are better or smarter than others sounds clichéd. The joke is usually on us when we deliberately seek to outsmart others. I recently came across a man who had manipulated the system so effectively that he has his cake and has been eating it too. Only because he makes the people around him feel superior. Every place has such smart people. We often find juniors outsmarting the egoistic seniors, class IV outsmarting their bosses and so on. Whether the act is cheating or outsmarting or manipulating depends on where we stand. If our perspective is of the boy in the story, then we are outsmarting. If it is of the politician, we are getting manipulated! The hunter and the hunted need not be permanent. The places can change. The anecdote is only serves a reminder for better introspection before we attempt to demean the others. Never sure who the victim is!
With this, we shall get back to the regular feature: Interesting clinical scenarios.
SMALL Vs BIG RV
We came across a toddler with TOF physiology with small Tricuspid valve annulus. The resultant RV was suboptimal in size. The decision was for a one-and-a-half ventricle correction. Since pulmonary annulus was small, the decision was to get a transannular patch done. How does such a physiology affect RV and Glenn shunt? Can the free PR be detrimental for RV function? Can the TAP take away the purpose of Glenn shunt in reducing RV preload? Even if RV sustains the load, can the increased preload of RV increase the eventual PA pressures and cause Glenn shunt to fail? If anyone has seen this combo working, please let us know the expected dynamics of post op status.
MASS(IVE) DECISION
A 10-year-old boy was admitted to our ICU with rapidly deteriorating LV function. He came in clear LVF. After the basic resuscitation, we did his echo. The LA and LV were dilated and LV EF was about 20%. Behind the LA, a dense, homogenous mass was visualised. The mass was compressing on the LA, deforming its contours. Moderate MR was seen. One of our colleagues noted a thickened mitral valve with restricted mobility of posterior leaflet. Some pericardial effusion added to confusion. Since the mass did not appear to have much effect on the ventricles and the history was acute, he suspected the diagnosis to be rheumatic, with associated mass. To add on, ASLO titres were positive, ESR and CRP were high. There was no clinical history suggestive of rheumatic fever. Still, the patient was started on steroids in suspicion of indolent rheumatic carditis in failure suggestive of rheumatic activity, along with supportive measures and antibiotics. The patient made a very good recovery on this, with EF increasing to 50% in 48 hours of therapy. We got a CT scan, which showed a homogenous mass behind the heart. The Hounsfield unit measurements favoured the diagnosis of lipoma. With the improvement of EF, repeat echo showed a normal motion of mitral valve, taking away the suspicion of RHD! We have stopped steroids and observing the child, with rest of the supportive treatments on. There are some pertinent questions to be answered. How did a slowly growing lipoma produce the mass effect all of a sudden? If no other super-added disease process is adding on, how do we explain the rapid deterioration in this boy? What post-operative histology can explain all the sequence of events? We are yet to get the surgery done for him due to infection. Once we get further answers, I shall brief you. But the rarity of the case is interesting. Lipomas in the mediastinum are not very frequent. That too, massive ones are very rare. We got just one case report in the literature. If any new waves of thought occur, please post them.
IMMUNE DECISION
How good are the screening tests for primary immune deficiency in a country like India? With such a massive population and rampant consanguinity, our numbers in primary immune deficiency must be enormous. However, no medical student with substantial number of years in paediatrics can recall many such children. Comparatively, our database on HIV is better, thanks to awareness and availability of diagnostic modalities. We had a 9-month-old with vegetations on both AV valves and aortic valve. The child had a significant history of abscess formation with IM injections. Our primary suspicion was immune deficiency. But, primary screening for that turned out to be negative. HIV screening in child and both parents was negative. We involved our hemato-oncologist into the picture, but the scenario did not become any clearer. Assays of immuneglobulins are just one part of the diagnosis. How about the complement function, leukocyte functional assay, receptor level abnormalities and others? Our fellows vehemently chased the diagnosis, but we are financially stressed. We did not want to burden the parents over massive expenditure on investigations. Has anyone come across cases like this anytime? How did you go about the diagnosis? Also of interest is the methodology to be followed in India if a suspected case of primary immune deficiency comes when the immune globulins are of normal levels. Please let us know your viewpoints on it.
UNGUARDED EBSTEINS
What is the DD for Ebsteins anomaly!? The question sounds a bit tough, as there can hardly be any. The picture of Ebsteins is so clear that even partially trained fetal echocardiographers vouch by the diagnosis. We had a baby with such an eventuality. It was reported as Ebsteins from outside. What we found surprised us. It was a case of unguarded tricuspid valve! The leaflets were in the right place with no displacement. But, none of them coapted with each other, leaving an unguarded opening to allow a free to-and-fro movement of the blood. The RA was enlarged and the moderator band looked thick and mobile, giving an impression of displaced STL. Is this condition common? It is possible to have missed this condition for Ebstiens. Please let me know if you have seen such entity earlier.
AGELESS REGURGITATION!
How would the pulmonary regurgitation behave with age in setting of absent pulmonic valve? We had a 24-year-old with TOF and absent pulmonic valve! His PR was minimal, but the PAs were huge. The latter findings suggest that the PR must have been there and significant. How to explain the minimal PR that we find now? No one could recall any patient of this age with such findings. Does the free/severe PR improve with age in absent pulmonic valve syndrome? If anyone has come across such a scenario, please let us know.
PEDIATRIC CARDIOLOGY PEARLS:
26. There is a sub-variant of Partial AV canal defect called Transitional type. In the transitional form of partial AVSD, there is aneurysmal replacement of a portion of the inlet ventricular septum, leaving a minimal VSD with a tricuspid pouch which usually obstructs any major shunting at the ventricular level. (Seward JB, Tajik AJ, Edwards WD, et al. Congenital heart disease. In: Two-Dimensional Echocardiographic Atlas. Vol. 1. New York: Springer-Verlag, 1987)
27. In 1973, when echocardiographic evaluation was not a part of diagnosis, it was suggested that the diameter of the ventricular septal defect compared to the diameter of the ascending aorta could be used in determining the prognosis. When the ratio was ≥ 0.8, this defect was considered large, was unlikely to spontaneously close, and the infant would have a large pulmonary blood flow and pulmonary artery hypertension. Although the determinants are changed with the advents of Echocardiography, few still consider this point as a significant one. (Rowe RD. In: Barratt- Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy. Diagnosis and Surgical Treatment. Proceedings of the Second International Symposium. Edinburgh: Churchill Livingstone, 1973 page 121)
28. In Atrial Septal defect, Eisenmenger reaction was found to be 7% in the first decade, 8% in the second decade, 10% in the third decade, and 11% in the fourth decade and beyond. (Cherian G, Uthaman CB, Durairaj M et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. American Heart Journal 1983 page 954)
29. The commonest congenital heart disease with right aortic arch is common arterial trunk (popularly called Truncus arteriosus) (Anderson RH, Thiene G. European Journal of Cardiothorac Surgery 1989 page 481)
30. In d-TGA newborns, increased numbers and size of pancreatic islet cells, and increased weight of the adrenal cortex are found. These findings in the pancreas and adrenal cortex are similar to those seen in infants of diabetic mothers and support the contention that the higher-than-usual glucose concentration in the descending aorta during fetal development may play a role (Naeye RL. Archives of Pathology 1966 page 412)
This brings us to the end of one more post. I had sent the emails to all the known followers last time. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
A couple of decades back in a small town lived a man famous for his intelligence. He was well read and respected. People used to come to him for discussing their problems. He would give them the advice, free of cost. In a way, he was popular as a wise man.
For obvious reasons, certain people in the town disliked him. They wanted to demean him. Since they would not be any match for him on a direct confrontation, they were seeking surrogate opportunities.
One day, someone told the man, “The local politician has been spreading the word that your son is an idiot. Do something”.
The man was surprised. His son was in 4th standard and far from idiocy! He was a clever boy, even though he never topped the class.
The man thought of getting the facts straight. He called his son to chamber and asked, “Do you know the local politician?”
“Yes”, the son answered with a twinkle in his eyes.
“He was saying something bad about you. Any tussle between you?”
“Oh, ignore him, Dad. He is not a smart man”
The man was surprised. The comment was too much for a boy of nine.
“You must not use such terms for elders. I have come to know that the man thinks you are an idiot. But I know that you are not. What makes him think so?”
The boy grinned. “The politician fellow has no good business. He sits with few of his friends everyday in the porch in front of his house. I have to go the same way to reach the school. On seeing, he calls me near him, keeps a 5 rupee coin in one palm and one rupee coin in the other. He asks me to pick up one coin. Once I pick up the coin, he laughs loudly and tells his friends: “Did I not tell you?” asks me to keep the coin for myself. Later all of them laugh again. This happens every day.”
The man was interested. “And what coin do you pick up?”
“Obviously, the one rupee coin”
The man was surprised. “I think you know that 5 is more than one?”
“Of course Dad, I know”
“Still you pick up the one rupee coin?”
The boy looked at his dad in surprise. He excused himself and left the room. He came back within a minute with a glass jar in his hand. The jar was three-fourth full of one-rupee coins.
“What is this?” the man demanded.
“This is to show who the idiot is!” the son laughed.
The man was still unsure whether he understood the situation correctly. “Explain” he told his son.
“It is wits versus stupidity. The moment I pick up the five-rupee coin, this joke would end. The politician has no other business than proving me wrong. I oblige. This will continue as long as I keep picking up the single rupee coin. I want to keep the game alive. Now, I want you to tell me whether 5 rupee coin is worth more or this jar.”
The man was taken aback. His son looked the smartest kid on the world to him at that moment.
The mere feeling that we are better or smarter than others sounds clichéd. The joke is usually on us when we deliberately seek to outsmart others. I recently came across a man who had manipulated the system so effectively that he has his cake and has been eating it too. Only because he makes the people around him feel superior. Every place has such smart people. We often find juniors outsmarting the egoistic seniors, class IV outsmarting their bosses and so on. Whether the act is cheating or outsmarting or manipulating depends on where we stand. If our perspective is of the boy in the story, then we are outsmarting. If it is of the politician, we are getting manipulated! The hunter and the hunted need not be permanent. The places can change. The anecdote is only serves a reminder for better introspection before we attempt to demean the others. Never sure who the victim is!
With this, we shall get back to the regular feature: Interesting clinical scenarios.
SMALL Vs BIG RV
We came across a toddler with TOF physiology with small Tricuspid valve annulus. The resultant RV was suboptimal in size. The decision was for a one-and-a-half ventricle correction. Since pulmonary annulus was small, the decision was to get a transannular patch done. How does such a physiology affect RV and Glenn shunt? Can the free PR be detrimental for RV function? Can the TAP take away the purpose of Glenn shunt in reducing RV preload? Even if RV sustains the load, can the increased preload of RV increase the eventual PA pressures and cause Glenn shunt to fail? If anyone has seen this combo working, please let us know the expected dynamics of post op status.
MASS(IVE) DECISION
A 10-year-old boy was admitted to our ICU with rapidly deteriorating LV function. He came in clear LVF. After the basic resuscitation, we did his echo. The LA and LV were dilated and LV EF was about 20%. Behind the LA, a dense, homogenous mass was visualised. The mass was compressing on the LA, deforming its contours. Moderate MR was seen. One of our colleagues noted a thickened mitral valve with restricted mobility of posterior leaflet. Some pericardial effusion added to confusion. Since the mass did not appear to have much effect on the ventricles and the history was acute, he suspected the diagnosis to be rheumatic, with associated mass. To add on, ASLO titres were positive, ESR and CRP were high. There was no clinical history suggestive of rheumatic fever. Still, the patient was started on steroids in suspicion of indolent rheumatic carditis in failure suggestive of rheumatic activity, along with supportive measures and antibiotics. The patient made a very good recovery on this, with EF increasing to 50% in 48 hours of therapy. We got a CT scan, which showed a homogenous mass behind the heart. The Hounsfield unit measurements favoured the diagnosis of lipoma. With the improvement of EF, repeat echo showed a normal motion of mitral valve, taking away the suspicion of RHD! We have stopped steroids and observing the child, with rest of the supportive treatments on. There are some pertinent questions to be answered. How did a slowly growing lipoma produce the mass effect all of a sudden? If no other super-added disease process is adding on, how do we explain the rapid deterioration in this boy? What post-operative histology can explain all the sequence of events? We are yet to get the surgery done for him due to infection. Once we get further answers, I shall brief you. But the rarity of the case is interesting. Lipomas in the mediastinum are not very frequent. That too, massive ones are very rare. We got just one case report in the literature. If any new waves of thought occur, please post them.
IMMUNE DECISION
How good are the screening tests for primary immune deficiency in a country like India? With such a massive population and rampant consanguinity, our numbers in primary immune deficiency must be enormous. However, no medical student with substantial number of years in paediatrics can recall many such children. Comparatively, our database on HIV is better, thanks to awareness and availability of diagnostic modalities. We had a 9-month-old with vegetations on both AV valves and aortic valve. The child had a significant history of abscess formation with IM injections. Our primary suspicion was immune deficiency. But, primary screening for that turned out to be negative. HIV screening in child and both parents was negative. We involved our hemato-oncologist into the picture, but the scenario did not become any clearer. Assays of immuneglobulins are just one part of the diagnosis. How about the complement function, leukocyte functional assay, receptor level abnormalities and others? Our fellows vehemently chased the diagnosis, but we are financially stressed. We did not want to burden the parents over massive expenditure on investigations. Has anyone come across cases like this anytime? How did you go about the diagnosis? Also of interest is the methodology to be followed in India if a suspected case of primary immune deficiency comes when the immune globulins are of normal levels. Please let us know your viewpoints on it.
UNGUARDED EBSTEINS
What is the DD for Ebsteins anomaly!? The question sounds a bit tough, as there can hardly be any. The picture of Ebsteins is so clear that even partially trained fetal echocardiographers vouch by the diagnosis. We had a baby with such an eventuality. It was reported as Ebsteins from outside. What we found surprised us. It was a case of unguarded tricuspid valve! The leaflets were in the right place with no displacement. But, none of them coapted with each other, leaving an unguarded opening to allow a free to-and-fro movement of the blood. The RA was enlarged and the moderator band looked thick and mobile, giving an impression of displaced STL. Is this condition common? It is possible to have missed this condition for Ebstiens. Please let me know if you have seen such entity earlier.
AGELESS REGURGITATION!
How would the pulmonary regurgitation behave with age in setting of absent pulmonic valve? We had a 24-year-old with TOF and absent pulmonic valve! His PR was minimal, but the PAs were huge. The latter findings suggest that the PR must have been there and significant. How to explain the minimal PR that we find now? No one could recall any patient of this age with such findings. Does the free/severe PR improve with age in absent pulmonic valve syndrome? If anyone has come across such a scenario, please let us know.
PEDIATRIC CARDIOLOGY PEARLS:
26. There is a sub-variant of Partial AV canal defect called Transitional type. In the transitional form of partial AVSD, there is aneurysmal replacement of a portion of the inlet ventricular septum, leaving a minimal VSD with a tricuspid pouch which usually obstructs any major shunting at the ventricular level. (Seward JB, Tajik AJ, Edwards WD, et al. Congenital heart disease. In: Two-Dimensional Echocardiographic Atlas. Vol. 1. New York: Springer-Verlag, 1987)
27. In 1973, when echocardiographic evaluation was not a part of diagnosis, it was suggested that the diameter of the ventricular septal defect compared to the diameter of the ascending aorta could be used in determining the prognosis. When the ratio was ≥ 0.8, this defect was considered large, was unlikely to spontaneously close, and the infant would have a large pulmonary blood flow and pulmonary artery hypertension. Although the determinants are changed with the advents of Echocardiography, few still consider this point as a significant one. (Rowe RD. In: Barratt- Boyes BG, Neutze JM, Harris EA, eds. Heart Disease in Infancy. Diagnosis and Surgical Treatment. Proceedings of the Second International Symposium. Edinburgh: Churchill Livingstone, 1973 page 121)
28. In Atrial Septal defect, Eisenmenger reaction was found to be 7% in the first decade, 8% in the second decade, 10% in the third decade, and 11% in the fourth decade and beyond. (Cherian G, Uthaman CB, Durairaj M et al. Pulmonary hypertension in isolated secundum atrial septal defect: high frequency in young patients. American Heart Journal 1983 page 954)
29. The commonest congenital heart disease with right aortic arch is common arterial trunk (popularly called Truncus arteriosus) (Anderson RH, Thiene G. European Journal of Cardiothorac Surgery 1989 page 481)
30. In d-TGA newborns, increased numbers and size of pancreatic islet cells, and increased weight of the adrenal cortex are found. These findings in the pancreas and adrenal cortex are similar to those seen in infants of diabetic mothers and support the contention that the higher-than-usual glucose concentration in the descending aorta during fetal development may play a role (Naeye RL. Archives of Pathology 1966 page 412)
This brings us to the end of one more post. I had sent the emails to all the known followers last time. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
ಶುಕ್ರವಾರ, ಜೂನ್ 4, 2010
Welcome to the readership. This is Dr Kiran inviting you to another post of blog. As previous, we shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, as we have been doing since past posts, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. The following story was told by one of the motivational speakers in a seminar. The speaker was author of multiple best-sellers and does motivational courses nowadays. Please go through this interesting anecdote.
A mother came with her adolescent son to a wise man. It was clear that the boy was brought there by emotional force! He looked uninterested.
“Sir”, the mother addressed the wise man. “This is my son. He is very intelligent, but not focused in life. I request you to give some advice to him”.
The wise man smiled at the boy, who frowned impatiently.
The wise man was indeed wise. He was a modern day Guru. He would not preach or advice atonement or punishments. His words were always more practical than many would think.
“Can we talk about something?” the wise man invited the boy into conversation.
The boy thought of leaving. He looked out of the window. A freshly asphalted road was seen. A small ball of tar was lying at a corner.
“Let us talk about that small ball of tar” the boy said, trying to ridicule the wise man.
The wise man did not get offended. “Can you please get that ball of tar?” he asked.
The boy felt happy. He wanted the “wise man” to give back nicely. He fetched the tar ball and gave it.
“How much do you think this tar ball would cost?” the wise man asked the boy.
“Cost? It is useless; worthless” the boy replied.
“These are basic hydrocarbaons, aren’t they? How much do you think its raw material would have costed?” the wise man had a conviction in his words this time.
“Boy, this fellow knows some chemistry too” the boy thought. He wanted to show off now. “May be, about ten bucks” he answered cautiously.
“Hmmm. Let us get few steps back. This is made of the same basic chemicals used to make plastics I believe?”
“Yes”, the boy was enthusiastic this time. “Vinyls and other polymers would have the basic molecular skeleton as this”
“High quality polymers are expensive. Is the same basic chemical composition applied there also?”
“That is correct. The hydrocarbons have same basic chemical structure. They can be sent through different pathways to obtain different substances. But the basic chemical skeleton remains the same”, the boy was feeling proud this time.
“I hear some of the finest and highest quality materials used in rockets and medical instruments can be prepared using the same chemical framework. I was told by a scientist that these substances cost almost Rs 10,000/- per meter” the wise man said with an innocent tone in his voice.
“You have heard him right. These are very high quality substances that need to be segregated at a very early stage of hydrocarbon processing. The channelization actually matters. Of course, high level of discipline and proper instrumentation along with appropriate environmental standards make all the difference”, the boy knew his stuff and was eager to show off.
“I was told mere ten grams of the basic substance can make high quality thread as long as 1,000 meters. Can it?”
“Of course. Such material used in critical processes may cost about Rs 1,000 a meter” the boy said with full vigor in his voice.
“So, you mean to say this useless, worthless ball of tar could have been something worth a million rupees only if were to get channelized at the beginning”, the voice of wise man was impassionate.
Silence. The boy did not say anything.
“Don’t you think the same process gets reflected in life?” the wise man saw into the eyes of the boy.
The boy bent his head. Words were struck in his throat. “Yes” he said slowly.
“Power, knowledge and intelligence may not reach where it deserves unless it gets a sense of direction and channelized into the proper pathway. It is left to you whether you wish to end up Rs1000 a meter or a waste mass of material at a deserted corner of road”, the wise man stopped.
The boy choked. The message was conveyed.
I remember one of my physics lecturers telling the class. “Be a vector. Your energies are nothing without the element of direction.” Not many of us could understand or appreciate what he told at that age. However, the words remained with me. I see a greater meaning now for the same. Every day, the same message gets extrapolated in many fronts. Even when it comes to management of heart problems, we often see how the small steps kept at early phases of disease process end up being great benefits for the child. Similarly, how small mistakes at the inception end up being a disaster for the patient. Appropriate channelization, whether in life or in any trifle looking setting, is indeed a big task. I felt like sharing this anecdote for the final punch it delivers and the resultant impact!
With this, it is time to get back to our regular feature: Interesting case scenarios:
INEVITABLE LAST MINUTE
We had 8-year-old with single ventricle physiology with IVC interruption and pulmonary atresia and intact IAS. How would the cath for single ventricle suitability help? We cannot enter the PA, cannot get the pulmonary venous wedge or have any way of measuring the PA pressures. Since it is a potential Kawashima, unless the data is clear, the decisions are pending. We contemplated this, yet went on with cath. Cath study did not spring any surprises and no clear data could be obtained. It was left to “on the table” decision making. Is there any other way of getting the data from cath? Please let me know your opinions on such scenarios.
POSSIBLY POSSIBLE!!
Is there a possibility of transposition of great arteries with {S,D,S} combination? In other words, can we have normally related great arteries in transposition diagnosis? The words sound paradoxical. However, we came across such a report done from outside. On repeating the echo, we found a DORV, NRGA, VSD, severe PAH. The commitment of PA was unclear and could be routed to RV or LV depending on how the patch is to be directed. When we put up the question on the possibility of outside report, we were surprised to hear that {S,D,S} in TGA is possible and reported. Has anyone come across this combination? If so, please send the details. We shall put it up in the subsequent posts.
CALCIUM DYNAMICS: IS PREDICTION POSSIBLE?
We had one 11-year-old girl, who had undergone RV to PA homograft conduit repair about 11 months back. The girl came back with calcification of homograft with severe obstruction. On the cath study, we found calcification on the VSD patch also! The surprise element was the rapidity with which the calcification had occurred and progressed. The surgical team introspected about the type of valve. They wanted to see the records to find out if it was an antibiotic prepared valve or a cryo preserved valve. Does it actually matter? Is there any way in which we can predict the calcification? Are there any observations regarding these issues? Please let me know your experiences on this.
NUMERICAL DILEMMAS
Sometimes, the initial assessment of a patient with high Qp and sever PAH shows clear operability on clinical assessment and basic investigations. However, the cath data on the same patient may not show the same picture. We had one 8-year-old of CCTGA, VSD with PAH. He was saturating 98%. However, the surgical team sought a cath study to ensure the anatomy. On the cath, the PVRI was 14.8 wood units! The surgery was ruled out due to the numbers. Many of us would have come across such situations. Is there a cut off for ordering additional investigations? If there is a disparity between the numerical values and clinical findings, doesn’t the latter get precedence? How to resolve such issues? If you have found any solution for these scenarios, please let us know.
COLLATERAL DAMAGE
We come across scenarios wherein the patients with single ventricle physiologies saturating well would be put on Glenn shunt for reducing the volume loads. We had a 2-year-old with single pump physiology saturating 92% in room air. He had good aortopulmonary collaterals. How should we go about dealing with these collaterals after the Glenn shunt? Do the saturations come down if we ligate the collaterals? Is a low SO2 after surgery acceptable? If we leave the collaterals, is the risk of overflow and volume load higher? The senior surgeons always maintain that the collaterals are self limiting and if jobless, they would involute by themselves. Is that true? If so, how long do they take? Is accepting low saturations better than risking overflow and volume load? The surgeons were reluctant due to issues related to approach. They suggested that we can coil the collaterals post op. What is more logical? If any other institute has any consensus on this issue, we would be interested to know their logic.
PEDIATRIC CARDIOLOGY PEARLS:
21. Arterial malformations have two vascular patterns: Arterio Venous Malformations (AVM) and Arterio Venous Fistulae (AVF). AVM (microfistulas) are multiple arterial feeders joined via a nidus to draining veins. AVF (macrofistulas) are direct shunts between large arterial and venous channels. (Avery JB, ed. Cardiovascular Pathology in Infants and Children. Philadelphia: WB Saunders, 1984 page277)
22. The relationship between development of aortic arch and migration of neural crest cells into the pharyngeal arches was given for first time in 1991 in avian embryo studies. (Kuratani S, Kirby ML. American Journal of Anatomy 1991 page 215)
23. Normally, there are two right and two left pulmonary veins. The most common variation is the presence of a single pulmonary vein on either the right or left side, with a prevalence of about 24% in anatomic studies. Contrary to the popular belief, the prevalence of a third pulmonary vein on either the right or left side is only 1.6% to 2%. (Healy JE Jr. Journal of Thoracic and Cardiovascular Surgery 1952 page 433)
24. The persistent Left SVC opens into coronary sinus and drains into Right atrium. Hence, the coronary sinus enlarges in presence of left SVC. This interferes with blood flow from the left atrium into the left ventricle. An increase in the magnitude of the left-to-right shunt at the atrial level was found in patients with secundum atrial septal defects (ASDs), persistent LSVC, and dilated coronary sinus. (Byerregaard P, Laursen HB. Acta Paediatrica Scandinevia 1980 page 105)
25. Left axis deviation in ECG is found in moderate VSD. However, large VSDs and equal ventricular pressures demonstrate right ventricular hypertrophy pattern in ECG. (van den Heuvel F, Timmers T, Hess J. British Heart Journal 1995 page 49)
This brings us to the end of one more post. I had sent the emails to all the known followers last time. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
A mother came with her adolescent son to a wise man. It was clear that the boy was brought there by emotional force! He looked uninterested.
“Sir”, the mother addressed the wise man. “This is my son. He is very intelligent, but not focused in life. I request you to give some advice to him”.
The wise man smiled at the boy, who frowned impatiently.
The wise man was indeed wise. He was a modern day Guru. He would not preach or advice atonement or punishments. His words were always more practical than many would think.
“Can we talk about something?” the wise man invited the boy into conversation.
The boy thought of leaving. He looked out of the window. A freshly asphalted road was seen. A small ball of tar was lying at a corner.
“Let us talk about that small ball of tar” the boy said, trying to ridicule the wise man.
The wise man did not get offended. “Can you please get that ball of tar?” he asked.
The boy felt happy. He wanted the “wise man” to give back nicely. He fetched the tar ball and gave it.
“How much do you think this tar ball would cost?” the wise man asked the boy.
“Cost? It is useless; worthless” the boy replied.
“These are basic hydrocarbaons, aren’t they? How much do you think its raw material would have costed?” the wise man had a conviction in his words this time.
“Boy, this fellow knows some chemistry too” the boy thought. He wanted to show off now. “May be, about ten bucks” he answered cautiously.
“Hmmm. Let us get few steps back. This is made of the same basic chemicals used to make plastics I believe?”
“Yes”, the boy was enthusiastic this time. “Vinyls and other polymers would have the basic molecular skeleton as this”
“High quality polymers are expensive. Is the same basic chemical composition applied there also?”
“That is correct. The hydrocarbons have same basic chemical structure. They can be sent through different pathways to obtain different substances. But the basic chemical skeleton remains the same”, the boy was feeling proud this time.
“I hear some of the finest and highest quality materials used in rockets and medical instruments can be prepared using the same chemical framework. I was told by a scientist that these substances cost almost Rs 10,000/- per meter” the wise man said with an innocent tone in his voice.
“You have heard him right. These are very high quality substances that need to be segregated at a very early stage of hydrocarbon processing. The channelization actually matters. Of course, high level of discipline and proper instrumentation along with appropriate environmental standards make all the difference”, the boy knew his stuff and was eager to show off.
“I was told mere ten grams of the basic substance can make high quality thread as long as 1,000 meters. Can it?”
“Of course. Such material used in critical processes may cost about Rs 1,000 a meter” the boy said with full vigor in his voice.
“So, you mean to say this useless, worthless ball of tar could have been something worth a million rupees only if were to get channelized at the beginning”, the voice of wise man was impassionate.
Silence. The boy did not say anything.
“Don’t you think the same process gets reflected in life?” the wise man saw into the eyes of the boy.
The boy bent his head. Words were struck in his throat. “Yes” he said slowly.
“Power, knowledge and intelligence may not reach where it deserves unless it gets a sense of direction and channelized into the proper pathway. It is left to you whether you wish to end up Rs1000 a meter or a waste mass of material at a deserted corner of road”, the wise man stopped.
The boy choked. The message was conveyed.
I remember one of my physics lecturers telling the class. “Be a vector. Your energies are nothing without the element of direction.” Not many of us could understand or appreciate what he told at that age. However, the words remained with me. I see a greater meaning now for the same. Every day, the same message gets extrapolated in many fronts. Even when it comes to management of heart problems, we often see how the small steps kept at early phases of disease process end up being great benefits for the child. Similarly, how small mistakes at the inception end up being a disaster for the patient. Appropriate channelization, whether in life or in any trifle looking setting, is indeed a big task. I felt like sharing this anecdote for the final punch it delivers and the resultant impact!
With this, it is time to get back to our regular feature: Interesting case scenarios:
INEVITABLE LAST MINUTE
We had 8-year-old with single ventricle physiology with IVC interruption and pulmonary atresia and intact IAS. How would the cath for single ventricle suitability help? We cannot enter the PA, cannot get the pulmonary venous wedge or have any way of measuring the PA pressures. Since it is a potential Kawashima, unless the data is clear, the decisions are pending. We contemplated this, yet went on with cath. Cath study did not spring any surprises and no clear data could be obtained. It was left to “on the table” decision making. Is there any other way of getting the data from cath? Please let me know your opinions on such scenarios.
POSSIBLY POSSIBLE!!
Is there a possibility of transposition of great arteries with {S,D,S} combination? In other words, can we have normally related great arteries in transposition diagnosis? The words sound paradoxical. However, we came across such a report done from outside. On repeating the echo, we found a DORV, NRGA, VSD, severe PAH. The commitment of PA was unclear and could be routed to RV or LV depending on how the patch is to be directed. When we put up the question on the possibility of outside report, we were surprised to hear that {S,D,S} in TGA is possible and reported. Has anyone come across this combination? If so, please send the details. We shall put it up in the subsequent posts.
CALCIUM DYNAMICS: IS PREDICTION POSSIBLE?
We had one 11-year-old girl, who had undergone RV to PA homograft conduit repair about 11 months back. The girl came back with calcification of homograft with severe obstruction. On the cath study, we found calcification on the VSD patch also! The surprise element was the rapidity with which the calcification had occurred and progressed. The surgical team introspected about the type of valve. They wanted to see the records to find out if it was an antibiotic prepared valve or a cryo preserved valve. Does it actually matter? Is there any way in which we can predict the calcification? Are there any observations regarding these issues? Please let me know your experiences on this.
NUMERICAL DILEMMAS
Sometimes, the initial assessment of a patient with high Qp and sever PAH shows clear operability on clinical assessment and basic investigations. However, the cath data on the same patient may not show the same picture. We had one 8-year-old of CCTGA, VSD with PAH. He was saturating 98%. However, the surgical team sought a cath study to ensure the anatomy. On the cath, the PVRI was 14.8 wood units! The surgery was ruled out due to the numbers. Many of us would have come across such situations. Is there a cut off for ordering additional investigations? If there is a disparity between the numerical values and clinical findings, doesn’t the latter get precedence? How to resolve such issues? If you have found any solution for these scenarios, please let us know.
COLLATERAL DAMAGE
We come across scenarios wherein the patients with single ventricle physiologies saturating well would be put on Glenn shunt for reducing the volume loads. We had a 2-year-old with single pump physiology saturating 92% in room air. He had good aortopulmonary collaterals. How should we go about dealing with these collaterals after the Glenn shunt? Do the saturations come down if we ligate the collaterals? Is a low SO2 after surgery acceptable? If we leave the collaterals, is the risk of overflow and volume load higher? The senior surgeons always maintain that the collaterals are self limiting and if jobless, they would involute by themselves. Is that true? If so, how long do they take? Is accepting low saturations better than risking overflow and volume load? The surgeons were reluctant due to issues related to approach. They suggested that we can coil the collaterals post op. What is more logical? If any other institute has any consensus on this issue, we would be interested to know their logic.
PEDIATRIC CARDIOLOGY PEARLS:
21. Arterial malformations have two vascular patterns: Arterio Venous Malformations (AVM) and Arterio Venous Fistulae (AVF). AVM (microfistulas) are multiple arterial feeders joined via a nidus to draining veins. AVF (macrofistulas) are direct shunts between large arterial and venous channels. (Avery JB, ed. Cardiovascular Pathology in Infants and Children. Philadelphia: WB Saunders, 1984 page277)
22. The relationship between development of aortic arch and migration of neural crest cells into the pharyngeal arches was given for first time in 1991 in avian embryo studies. (Kuratani S, Kirby ML. American Journal of Anatomy 1991 page 215)
23. Normally, there are two right and two left pulmonary veins. The most common variation is the presence of a single pulmonary vein on either the right or left side, with a prevalence of about 24% in anatomic studies. Contrary to the popular belief, the prevalence of a third pulmonary vein on either the right or left side is only 1.6% to 2%. (Healy JE Jr. Journal of Thoracic and Cardiovascular Surgery 1952 page 433)
24. The persistent Left SVC opens into coronary sinus and drains into Right atrium. Hence, the coronary sinus enlarges in presence of left SVC. This interferes with blood flow from the left atrium into the left ventricle. An increase in the magnitude of the left-to-right shunt at the atrial level was found in patients with secundum atrial septal defects (ASDs), persistent LSVC, and dilated coronary sinus. (Byerregaard P, Laursen HB. Acta Paediatrica Scandinevia 1980 page 105)
25. Left axis deviation in ECG is found in moderate VSD. However, large VSDs and equal ventricular pressures demonstrate right ventricular hypertrophy pattern in ECG. (van den Heuvel F, Timmers T, Hess J. British Heart Journal 1995 page 49)
This brings us to the end of one more post. I had sent the emails to all the known followers last time. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
ಶನಿವಾರ, ಮೇ 15, 2010
Hello everyone. This is Dr Kiran welcoming you to another post of blog. We shall see few interesting learning scenarios, few pearls of pediatric cardiology. Before that, let us see a small anecdote, which may carry a greater meaning than what it superficially denotes. This particular anecdote was put up in newspaper article. I happened to call the author and found it to be semiautobiographical. I found this worth sharing. Please go through.
Rakesh was 18 years old. He was never much interested in studies. He always had an ear for music, although he was a lousy singer by himself. He would attend many concerts at his place whenever he found an opportunity, setting everything else aside.
Rakesh’s father owned a small departmental store. They were not super-rich, but were well to do. Father’s ambition was to see a future for his son. As the son was never interested in future studies and had a minimal chances for an office job, his father had intentions of putting him into their business. However, Rakesh never showed any interest in business.
Rakesh had many friends who were equally interested in music. They used to attend the concerts together. Most of them were from middle class families and were not as well to do as Rakesh.
One day the father found Rakesh sitting alone in the living room. “What’s the matter?”, he enquired.
“Nothing great”, replied Rakesh and showed a newspaper item to his dad.
It was about a music concert at a place about 1000 km from their place.
“This man is great. I wish I could have attended his concert”, said Rakesh in a low tone.
His father saw the newspaper item for few moments. “Mind if I join you for the concert?” he asked his son.
Rakesh could not believe his ears. “Really? By all means” he said. “My accommodation problem also solved!” he said silently to himself.
They had a great journey by flight; stayed in a good lodge. They attended the concert. Rakesh was thrilled. His father just sat next to Rakesh throughout the concert.
They were back in the lodge and started packing. They were due for departure early morning.
“I don’t know how to thank you, Dad”, said Rakesh. “I never thought you enjoyed music. All my friends wanted to attend this concert, but you made it for me.”
“Two things” his father said. “First: I don’t enjoy music. But I do enjoy you enjoying it. I wanted to see you happy. So, I joined you all this distance. I am happy that you are happy.”
Rakesh was stunned. His father had driven the point quite well.
“Second”, his father continued. “You said lot of your friends wanted to attend this concert. None could. Largely because they could not afford the travel, stay and all the other expenses.”
Rakesh listened attentively. His father continued.
“In life, both the things should be balanced. One should have a passion: music as in your case. Also, everyone should have a profession which can make the passion possible and reachable. I could make your passion possible because I have a profession which can afford it. Lack of the second would make the first redundant. That is what has happened to your friends.”
Rakesh could appreciate what his father said. His father continued.
“I do not specify the profession. It can be anything of your choice. Government job, private firms, business, contract, whatever it may be. One should have an income enough to sustain the needs of himself and his dependents. At the same time, the needs should not extend the stretchable limits of income. This is the balance one has to achieve. If you wanted to attend a concert somewhere abroad, I probably would not have afforded.”
Rakesh appreciated the genuine tone of his father’s words. He did not feel any preaching. His father sounded more practical than he ever had been.
“In your case, I can give you an option to take care of our store. It is purely your wish. If you want to continue to study, please do so. If you want to find an office job, try it. But, don’t stay stale. Keep doing something constructive or potentially constructive. Early birds usually have an advantage.”
Rakesh wanted to listen, but his dad stopped there. There was some silence. Without many words, they completed packing.
They returned home. That evening Rakesh reached their departmental store. He waited till his dad completed a transaction with a customer. “Yes, Rakesh”, his father asked.
“Dad”, Rakesh replied. “Can you teach me how our store functions? I want to join as a regular salesperson and learn the trade.”
Tears of joy filled the eyes of his father.
If there is anything that we in medical profession miss, then it is the passion for non-medical issues. I have rarely seen medical professionals who have kept the other passions intact. I had heard the story of a famous neurosurgeon who cried on the day of his retirement for not having continued his passion for violin. Also, I knew a medical student who tried to seriously pursue his passion for painting along with medicine and failed in both. It is preferable to have a mature person advising us on how to balance both, but not everyone may find one. This story may tell us something we have already learnt many a times. Yet, it may be worth the pondering.
With this, we shall get back to the interesting learning scenarios.
ALTERNATE PATHWAY
In scientific temper, it is very difficult to accept something without adequately exploring all the possibilities. We had a 3-month-old with Tricuspid atresia 1A. There was only a restrictive PDA. However, the pulmonary venous return was much more impressive than what was suggested by PDA. But on echo, we could not locate any other shunt. A continuous murmur suggested the presence of collaterals, but the same could not be visualised on echo. Since the saturations were less, it was decided to go for a BT shunt. On the table, a coronary fistula was found to distal PA! This explained the picture clearly. Sometimes there is more to the patient than what meets the echo eyes! Please let me know if you have seen similar pictures.
ALL OR NONE?!
We had a 4-year-old with TGA, multiple VSDs, moderate to severe PS with L-malposed great arteries, saturating 85% in room air. On cath, the data appeared to be suitable for ASO with Rastelli repair. The additional VSDs were an issue. The surgical team felt that as many VSDs should be closed as possible. Even then, few VSDs may remain and the final procedure would be a palliative ASO. We were thinking whether such a heroic procedure is worth it. If some VSDs remain, they themselves may be a major cause for concern. Unless we can ensure complete closure of all VSDs, ASO in L-malposed set up may not be worth all the high risk procedure. With the balanced physiology the patient had, one of the contemplations was to leave him alone. What is the opinion of the readership? Let us know your take on this.
RISK Vs RISKIER
We often face problems in those age groups that we are very comfortable with. We had a 21-year-old with cTGA, VSD, PS in the setting of situs inversus. He was saturating 87% in room air. Cath data showed a routable VSD. But his ventricular EDP was 20 mmHg. It was presumed that the raised EDP was secondary to chronic hypoxia and cyanosis. One option was to do a double switch – Senning with Rastelli. The caveat was about the success of Senning in high EDPs. The other option was to leave him as he is now. This may lead to further dysfunction of ventricles with time. Is the option of doing the surgery in situs inversus and its outcome riskier than the medical follow up? What is the natural history of untreated cTGA, VSD, PS? What the average progress of the ventricular dysfunction in such cases? Is there an index for predicting the progress? Such questions remain unanswered. Neither the surgical team nor our team could find an answer for these questions. If anyone has any experiences in these issues, please let us know.
DOUBLE JEOPERDY
One of the inherent weaknesses of the human mind is to fit the findings to a diagnosis that is known! Sometimes, when all the findings are somehow not fitting into a clear picture, we presume that it is a variant of a known diagnosis and try to fit it. Same happened to me one of these days. We had a 9-year-old coming to us with left upper limb hypertension and low BP on right along with absent femorals. I could see a right arch which was narrow at isthmus and the adjacent subclavian showing turbulence at the origin. Since it was a right arch and the echo windows were not to boast of, I presumed a mirror imaging and gave a diagnosis of possible Takayasu disease. However, the cardiac CT undid the actual diagnosis. It was right arch with normal branching, coarctation of aorta at isthmus with aberrant origin of right subclavian artery from the post stenotic segment with the diverticulum of Kommarel. The combination could easily explain the clinical picture. With 2 vessels showing the turbulence with the disparity of pulses, I was lured into making a diagnosis of Takayasu disease neglecting all other markers!! In retrospect, I feel that if I had spent some more time in visualising the arch, I would probably had better chances of making the correct diagnosis. It was a lesson re-learnt. I don’t know how long it will stay! Let me know if you were also lured into making any such diagnosis which caused certain regret on retrospect.
SHUNT IN REGURGITATION
This issue has always troubled me, but did not have a platform to discuss. I am presenting this before everyone for the individual opinion. If anyone happens to find a literature evidence, please support it. The question is: What happens to Qp/Qs in cases of VSD with MR? We had a 6-year-old with complete AV canal defect saturating 92%. He had severe AV valve regurgitation. His calculated Qp/Qs was 1.15:1. Based on the number, he was considered as inoperable. The question was: if there were to be no AVVR, would his Qp/Qs be different? Doesn’t VSD get underestimated in the presence of AVVR? Since AVVR is PVRI independent, the VSD shunt would definitely be influenced by AVVR. Since the AVVR is surgically correctable in this case, the logic of refusing the surgery based on original Qp/Qs correct? The original question still remains. Is the Qp/Qs affected by MR? Or, is the regurgitant volume remains the same and gets nullified in the final equation? Please enlighten.
PEDIATRIC CARDIOLOGY PEARLS:
16. The recommended age for the closure of ASD is around 4 years. This was based on a study in 1983 by Cocherham et al in 87 children with ASD. It is followed as a matter of fact in all places. (Cockerham JT, Martin TC, Gutierrez FR, et al. American Journal of Cardiology 1983 page 1267)
17. An interesting term called double outlet right atrium was introduced by Horiuchi et al in 1976. It involves a primum defect with deviation of interatrial septum to the left. In true sense, it is only AV Canal defect. (Horiuchi T, Saji K, Osuka Y, et al. Journal of Cardiovascular Surgery (Torino) 1976 page 157)
18. Patients with outlet ventricular septal defects usually have deficiency of muscular or fibrous support below the aortic valve with herniation of the right coronary leaflet through the VSD. This leads to progressive aortic valve regurgitation with time. Hence, it is important to recognise this at the earliest and do a surgical correction to avoid any damage to aortic valve. (Van Praagh R, McNamara JJ. American Heart Journal 1968 page 604)
19. The continuous murmur of PDA has the highest number of descriptive names! It was originally described by Gibson in 1900 as having late systolic accentuation and continuation through the second sound into diastole. He, incidentally, never used the term continuous! (Gibson GA. Persistence of the arterial duct and its diagnosis. Edinburgh Medical Journal 1900 page 1)
20. Bland White Garland syndrome refers to ALCAPA. It goes by the names of 3 physicians who described it in 1933. Few have confused it for a bland patient, who looks white because of ischemia and ready for a garland on passing away! (Bland EF, White PD, Garland J. American Heart Journal 1933 page 787)
This brings us to the end of another post. My good friend Dr Prem Alva suggested informing all the followers by email on every update. Sounds practical. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
Rakesh was 18 years old. He was never much interested in studies. He always had an ear for music, although he was a lousy singer by himself. He would attend many concerts at his place whenever he found an opportunity, setting everything else aside.
Rakesh’s father owned a small departmental store. They were not super-rich, but were well to do. Father’s ambition was to see a future for his son. As the son was never interested in future studies and had a minimal chances for an office job, his father had intentions of putting him into their business. However, Rakesh never showed any interest in business.
Rakesh had many friends who were equally interested in music. They used to attend the concerts together. Most of them were from middle class families and were not as well to do as Rakesh.
One day the father found Rakesh sitting alone in the living room. “What’s the matter?”, he enquired.
“Nothing great”, replied Rakesh and showed a newspaper item to his dad.
It was about a music concert at a place about 1000 km from their place.
“This man is great. I wish I could have attended his concert”, said Rakesh in a low tone.
His father saw the newspaper item for few moments. “Mind if I join you for the concert?” he asked his son.
Rakesh could not believe his ears. “Really? By all means” he said. “My accommodation problem also solved!” he said silently to himself.
They had a great journey by flight; stayed in a good lodge. They attended the concert. Rakesh was thrilled. His father just sat next to Rakesh throughout the concert.
They were back in the lodge and started packing. They were due for departure early morning.
“I don’t know how to thank you, Dad”, said Rakesh. “I never thought you enjoyed music. All my friends wanted to attend this concert, but you made it for me.”
“Two things” his father said. “First: I don’t enjoy music. But I do enjoy you enjoying it. I wanted to see you happy. So, I joined you all this distance. I am happy that you are happy.”
Rakesh was stunned. His father had driven the point quite well.
“Second”, his father continued. “You said lot of your friends wanted to attend this concert. None could. Largely because they could not afford the travel, stay and all the other expenses.”
Rakesh listened attentively. His father continued.
“In life, both the things should be balanced. One should have a passion: music as in your case. Also, everyone should have a profession which can make the passion possible and reachable. I could make your passion possible because I have a profession which can afford it. Lack of the second would make the first redundant. That is what has happened to your friends.”
Rakesh could appreciate what his father said. His father continued.
“I do not specify the profession. It can be anything of your choice. Government job, private firms, business, contract, whatever it may be. One should have an income enough to sustain the needs of himself and his dependents. At the same time, the needs should not extend the stretchable limits of income. This is the balance one has to achieve. If you wanted to attend a concert somewhere abroad, I probably would not have afforded.”
Rakesh appreciated the genuine tone of his father’s words. He did not feel any preaching. His father sounded more practical than he ever had been.
“In your case, I can give you an option to take care of our store. It is purely your wish. If you want to continue to study, please do so. If you want to find an office job, try it. But, don’t stay stale. Keep doing something constructive or potentially constructive. Early birds usually have an advantage.”
Rakesh wanted to listen, but his dad stopped there. There was some silence. Without many words, they completed packing.
They returned home. That evening Rakesh reached their departmental store. He waited till his dad completed a transaction with a customer. “Yes, Rakesh”, his father asked.
“Dad”, Rakesh replied. “Can you teach me how our store functions? I want to join as a regular salesperson and learn the trade.”
Tears of joy filled the eyes of his father.
If there is anything that we in medical profession miss, then it is the passion for non-medical issues. I have rarely seen medical professionals who have kept the other passions intact. I had heard the story of a famous neurosurgeon who cried on the day of his retirement for not having continued his passion for violin. Also, I knew a medical student who tried to seriously pursue his passion for painting along with medicine and failed in both. It is preferable to have a mature person advising us on how to balance both, but not everyone may find one. This story may tell us something we have already learnt many a times. Yet, it may be worth the pondering.
With this, we shall get back to the interesting learning scenarios.
ALTERNATE PATHWAY
In scientific temper, it is very difficult to accept something without adequately exploring all the possibilities. We had a 3-month-old with Tricuspid atresia 1A. There was only a restrictive PDA. However, the pulmonary venous return was much more impressive than what was suggested by PDA. But on echo, we could not locate any other shunt. A continuous murmur suggested the presence of collaterals, but the same could not be visualised on echo. Since the saturations were less, it was decided to go for a BT shunt. On the table, a coronary fistula was found to distal PA! This explained the picture clearly. Sometimes there is more to the patient than what meets the echo eyes! Please let me know if you have seen similar pictures.
ALL OR NONE?!
We had a 4-year-old with TGA, multiple VSDs, moderate to severe PS with L-malposed great arteries, saturating 85% in room air. On cath, the data appeared to be suitable for ASO with Rastelli repair. The additional VSDs were an issue. The surgical team felt that as many VSDs should be closed as possible. Even then, few VSDs may remain and the final procedure would be a palliative ASO. We were thinking whether such a heroic procedure is worth it. If some VSDs remain, they themselves may be a major cause for concern. Unless we can ensure complete closure of all VSDs, ASO in L-malposed set up may not be worth all the high risk procedure. With the balanced physiology the patient had, one of the contemplations was to leave him alone. What is the opinion of the readership? Let us know your take on this.
RISK Vs RISKIER
We often face problems in those age groups that we are very comfortable with. We had a 21-year-old with cTGA, VSD, PS in the setting of situs inversus. He was saturating 87% in room air. Cath data showed a routable VSD. But his ventricular EDP was 20 mmHg. It was presumed that the raised EDP was secondary to chronic hypoxia and cyanosis. One option was to do a double switch – Senning with Rastelli. The caveat was about the success of Senning in high EDPs. The other option was to leave him as he is now. This may lead to further dysfunction of ventricles with time. Is the option of doing the surgery in situs inversus and its outcome riskier than the medical follow up? What is the natural history of untreated cTGA, VSD, PS? What the average progress of the ventricular dysfunction in such cases? Is there an index for predicting the progress? Such questions remain unanswered. Neither the surgical team nor our team could find an answer for these questions. If anyone has any experiences in these issues, please let us know.
DOUBLE JEOPERDY
One of the inherent weaknesses of the human mind is to fit the findings to a diagnosis that is known! Sometimes, when all the findings are somehow not fitting into a clear picture, we presume that it is a variant of a known diagnosis and try to fit it. Same happened to me one of these days. We had a 9-year-old coming to us with left upper limb hypertension and low BP on right along with absent femorals. I could see a right arch which was narrow at isthmus and the adjacent subclavian showing turbulence at the origin. Since it was a right arch and the echo windows were not to boast of, I presumed a mirror imaging and gave a diagnosis of possible Takayasu disease. However, the cardiac CT undid the actual diagnosis. It was right arch with normal branching, coarctation of aorta at isthmus with aberrant origin of right subclavian artery from the post stenotic segment with the diverticulum of Kommarel. The combination could easily explain the clinical picture. With 2 vessels showing the turbulence with the disparity of pulses, I was lured into making a diagnosis of Takayasu disease neglecting all other markers!! In retrospect, I feel that if I had spent some more time in visualising the arch, I would probably had better chances of making the correct diagnosis. It was a lesson re-learnt. I don’t know how long it will stay! Let me know if you were also lured into making any such diagnosis which caused certain regret on retrospect.
SHUNT IN REGURGITATION
This issue has always troubled me, but did not have a platform to discuss. I am presenting this before everyone for the individual opinion. If anyone happens to find a literature evidence, please support it. The question is: What happens to Qp/Qs in cases of VSD with MR? We had a 6-year-old with complete AV canal defect saturating 92%. He had severe AV valve regurgitation. His calculated Qp/Qs was 1.15:1. Based on the number, he was considered as inoperable. The question was: if there were to be no AVVR, would his Qp/Qs be different? Doesn’t VSD get underestimated in the presence of AVVR? Since AVVR is PVRI independent, the VSD shunt would definitely be influenced by AVVR. Since the AVVR is surgically correctable in this case, the logic of refusing the surgery based on original Qp/Qs correct? The original question still remains. Is the Qp/Qs affected by MR? Or, is the regurgitant volume remains the same and gets nullified in the final equation? Please enlighten.
PEDIATRIC CARDIOLOGY PEARLS:
16. The recommended age for the closure of ASD is around 4 years. This was based on a study in 1983 by Cocherham et al in 87 children with ASD. It is followed as a matter of fact in all places. (Cockerham JT, Martin TC, Gutierrez FR, et al. American Journal of Cardiology 1983 page 1267)
17. An interesting term called double outlet right atrium was introduced by Horiuchi et al in 1976. It involves a primum defect with deviation of interatrial septum to the left. In true sense, it is only AV Canal defect. (Horiuchi T, Saji K, Osuka Y, et al. Journal of Cardiovascular Surgery (Torino) 1976 page 157)
18. Patients with outlet ventricular septal defects usually have deficiency of muscular or fibrous support below the aortic valve with herniation of the right coronary leaflet through the VSD. This leads to progressive aortic valve regurgitation with time. Hence, it is important to recognise this at the earliest and do a surgical correction to avoid any damage to aortic valve. (Van Praagh R, McNamara JJ. American Heart Journal 1968 page 604)
19. The continuous murmur of PDA has the highest number of descriptive names! It was originally described by Gibson in 1900 as having late systolic accentuation and continuation through the second sound into diastole. He, incidentally, never used the term continuous! (Gibson GA. Persistence of the arterial duct and its diagnosis. Edinburgh Medical Journal 1900 page 1)
20. Bland White Garland syndrome refers to ALCAPA. It goes by the names of 3 physicians who described it in 1933. Few have confused it for a bland patient, who looks white because of ischemia and ready for a garland on passing away! (Bland EF, White PD, Garland J. American Heart Journal 1933 page 787)
This brings us to the end of another post. My good friend Dr Prem Alva suggested informing all the followers by email on every update. Sounds practical. 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. Also, send your feedbacks by email or via the comments section.
Regards
KIran
ಭಾನುವಾರ, ಮೇ 9, 2010
Hello all. This is Dr Kiran, welcoming you to the present session of blog. We were in the process of learning greater meaning of few anecdotes. The present anecdote was picked up from a newspaper article. Please go through.
Mr Iyer was a busy man. He worked for a MNC. Clock and calendars did not have any role in his life. He would often work for days together without coming home. He was known for his rigid stands, no-nonsense approach and upright decisions. When some lesser mortals made the mistake of asking about his salary, he would reply, “Rs.1000 per hour” to prevent them from talking any further.
Mr Iyer had a relatively big test the next day. He was supposed to give a business presentation to one of the global biggies. True to his meticulous self, he had done everything to perfection. He wanted to make sure that the things are alright. He took the previous day off and stayed home to fine-tune his presentation.
Mr Iyer’s son was a bubbly 5-year-old. People described him of having inherited his father’s intelligence. The son was elated to see his dad home that day and refused to go to school.
Mr Iyer did not heed much importance to this issue. He wanted peace of mind. He thought that losing his temper on anything may affect his performance the next day. He just let his son stay back.
The scene was not as simple as Mr Iyer thought. His son came down to the dad every two minutes and kept talking to him. Mr Iyer tried his best to calm himself, but his son was too insisting.
“Don’t you see that I am working on a presentation? This is important. Tell me what you want and let go of me” he tried to negotiate from his son.
The son was shocked for a while. He left the room, but returned 5 minutes later. “Dad”, he cried. “Can I have Rs 100/- from you please?”
On other times, Mr Iyer would have asked for reason, but this day was unlike that. He brought out his wallet and threw a Rs 100/- bill on his son. “Take this and leave” he demanded.
The boy picked up the cash from the floor and left. He returned in next five minutes. “Dad”, he said softly this time.
Mr Iyer’s temper was rising. He was getting annoyed. He grinded his teeth and asked in a stern voice, “What now?” “Please see” the son demanded.
Mr Iyer decided that unless some force is applied, this disturbance would persist. He got up from his chair and was about to thrash his son, when he noticed the clutched fist of his son held towards him.
“What is in your hand?” Mr Iyer asked angrily.
His son slowly unclenched his fist. There were few notes of various designations in the hand.
Mr Iyer was perplexed. “What are you doing? What do you want?” he asked in the same tone of annoyance.
“I opened my piggy bank. It had Rs 400/- I have taken Rs 100/- from you.”
“So?” the tone of Mr Iyer was mellowing down.
“I have heard you telling many uncles that you earn Rs 1000/- per hour.”
Mr Iyer was silent this time. His son continued.
“I have Rs 500/- with me. Can you please take this and play with me for half an hour?”
Mr Iyer went back to his laptop and shut it down. He played and spent time with his son the rest of the day. Mr Iyer was so much pleased with himself that his presentation the next day was applauded by everyone as the best he had done till then.
Very often, we do not understand the value small things. What we perceive as a small issue may really be a big one for the others. Our few minutes may be what the opposite person desperately needs. It is true with our family members and patients too. Often, big-wigs of the hospitals find it futile to find any time for their subordinates and patient attendants. I have seen busy practitioners starting their day around 5 pm and going home back by midnight. They are proud of their time management skills and claim that they stretch a couple of hours sleep in their cars during the travel. Few also say that their kids would not have woken up when they leave the house and would have slept well by the time they reach home. One of our consultants was very angry that he had to apply leave for a day to attend his son’s school day, as the boy was insisting on it a lot. It is very common in medical profession to have such scenarios. What majority does usually become a rule and makes an easy way others to follow. Doctors who try to defy the rule are termed “lazy”, “useless”, “waste body” and so on. It may be prudent to understand what the time management actually stands for before deciding on to invest on it. This small anecdote can open up lot of these issues pertinent to each of us in its own way.
With this, let us go back to the interesting learning scenarios of this post.
PROBLEM WITH WINDOWS
We had a situation which took lot of cerebral exercise. This 11-year-old boy had undergone closure of AP Window and PDA few years back. He came back to us with exertional dyspneoa. On echo, we reported him as possible residual PDA as suggested by the location of jet. However, on cath study, the outcome was a residual AP window. The data showed operability on oxygen study. During the cath meeting, our senior surgeon had a different opinion. He felt that the cause of this residual APW after these many years might be due to high PVRI per se and the residual opening is actually a pop-off. In his opinion, such lesions should be left alone if the symptoms are not much pressing. But the data we had did not suggest the same. Hence, we tried to attempt device closure of the lesion. We initially tried a VSD device, but it slipped. After few days, we tried a PDA device unsuccessfully. Now, we may not attempt the non-surgical ways anymore. What is the opinion of the readership on this? We shall keep you informed on the progress of this boy. Let me know your ideas on this issue.
PULMONARY CONTRIBUTION
We often see Qp/Qs in cath studies that are too high to believe. Yet, the pressure from dilated pulmonary arteries on the bronchi is not too high to compress the respiratory tract. We had a 8-month-old blue boy with large ASD shunting bidirectional and multiple muscular VSDs shunting right to left. The CHD lesions could not explain the clinical scenario. We found reduced air entry on the left lung. The chest radiograph showed collapsed left lung. The cardiac CT showed a completely collapsed left bronchus with collapsed left lung and a dilated left pulmonary artery adjacent to the bronchus. How to chronalize the cause-effect relationship? The surgical team felt that the dilated PA must have compressed the Left bronchus and the resultant lung collapse must have contributed to the PVRI causing right to left shunt. But, haven’t we seen ample number of large ASDs? How many times do we recall such a picture happening? Our logic was different. There might be an inherent defect in the left bronchus, which might have collapsed early due to added pressure by the LPA. Who is correct? We asked for pediatric surgical opinion for which a bronchoscopy for learning about the status of left bronchus. But the bronchoscopy was not much helpful. We were not clear on how to proceed. I hope the readership can also participate in this cerebral exercise for a while and come out with their suggestion.
BALLOON DECISIONS
How far is the balloon occlusion helpful in decision making of PDA operability? The technique or the interpretation does not seem to be standardized. We had an adult lady with a decent PDA shunting left to right. The balloon occlusion data was not helpful either way. We still went ahead with the device closure based on clinical and echo data. It turned out to be successful. The patient went home well. If we had gone by the cath data alone, we probably would have not touched the patient. Taking the entire picture, the cath data on balloon occlusion was invalid. Can the readership inform any published data on the proper balloon occlusion technique and interpretation? Please let me know.
TRACING THE PROBLEM
It was a nice question which we never had thought prior. The LV and RV tracings in the cath are very characteristic and reproducible. What causes the difference in the morphology of the tracing pattern? The question came up when one of the students put up this question in an e-class. “What does the LV tracing look like in a TGA?” Logical answer may be “like an RV tracing”. But, what is the correct answer? Please let me know the answer with references.
DISGUISING AS TAPVC
We had a 2-month-old who came to us with an echo report from outside as TAPVC. On the echo, we were surprised with the left to right shunting across the ASD. The left atrium looked very small with a normal mitral valve. The pulmonary veins were seen draining into the left atrium. Why was the LA small? On a close look, we found a bleak line within the presumed LA outside which no colour percolated. We decided that the echo free space is likely to be a cyst. Cardiac CT defined the mass to be cystic. On the surgical table, the cystic lesion was confirmed. There was no TAPVC. The smallish LA was secondary to an external compression by a cystic mass. It was only because of a machine with good resolution that we could pick up the diagnosis. I wonder what I could have done with a suboptimal machine that I am usually handed with at peripheral centres! If the readership has come across any similar events, please let us know.
PEDIATRIC CARDIOLOGY PEARLS:
11. The embrtological sequence of atrial septation is one of the most interesting understandings for both students and examiners. This hypothesis has been successful in explaining the abnormalities of atrial sepatations. This sequence of events was explained for the first time by van Mierop in 1976. Even today, we follow the same with few minor modifications. (Van Mierop LHS. In: Feldt RH, McGoon DC, Ongley PA, et al., eds. Atrioventricular Canal Defects. WB Saunders publications, 1976: page 12)
12. Children with Down syndrome are more likely to have complete AVSD than children without Down syndrome. They are also more likely to have associated tetralogy of Fallot (Vet TW, Ottenkamp. J in American Journal of Diseases in Children 1989, page 1362)
13. VSD happens to have most variants of classification. The most accepted version was published by Soto et al in 1980. In this classification, the ventricular septum is considered to have four components: An inlet septum separating the mitral and tricuspid valves; a trabecular septum, which extends from the attachments of the tricuspid leaflets outward to the apex and upward to the crista supraventricularis; the smooth-walled outlet or infundibular septum, which extends from the crista to the pulmonary valve; and the membranous septum, which is relatively small and is usually divided into two parts by the septal leaflet of the tricuspid valve. Each zone has defects going by their generic name. (Soto B, Becker AE, Moulaert AJ, et al, British Heart Journal 1980, page 33)
14. The incidence of PDA is about 30 times greater at high altitude (4,500 to 5,000 m) than at sea level (Alzamora-Castro V, Battilana G, Abugattas R, et al. American Journal of Cardiology 1960, page 761)
15. In Tetralogy of Fallot, about 40% of patients have an abnormally long, large conus artery from the right coronary artery that supplies a significant mass of myocardium. In 4% to 5%, the left anterior descending coronary artery arises from the right coronary artery and passes across the right ventricular outflow tract (RVOT). The resultant abnormal anatomy leads to compromises for surgical repair in the region of RVOT and pulmonary annulus. Hence, if the echo does not pick up the anomaly, one needs cath for proper surgical planning. (Fellows KE, Freed MD, Keane JF, et al. Circulation journal 1975, page 561)
With this, the present post ends. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am planning to add few more things to the new segment. Please ask your general pediatric friends about the blog and get their feedback on what would their need be.
Regards
Kiran
Mr Iyer was a busy man. He worked for a MNC. Clock and calendars did not have any role in his life. He would often work for days together without coming home. He was known for his rigid stands, no-nonsense approach and upright decisions. When some lesser mortals made the mistake of asking about his salary, he would reply, “Rs.1000 per hour” to prevent them from talking any further.
Mr Iyer had a relatively big test the next day. He was supposed to give a business presentation to one of the global biggies. True to his meticulous self, he had done everything to perfection. He wanted to make sure that the things are alright. He took the previous day off and stayed home to fine-tune his presentation.
Mr Iyer’s son was a bubbly 5-year-old. People described him of having inherited his father’s intelligence. The son was elated to see his dad home that day and refused to go to school.
Mr Iyer did not heed much importance to this issue. He wanted peace of mind. He thought that losing his temper on anything may affect his performance the next day. He just let his son stay back.
The scene was not as simple as Mr Iyer thought. His son came down to the dad every two minutes and kept talking to him. Mr Iyer tried his best to calm himself, but his son was too insisting.
“Don’t you see that I am working on a presentation? This is important. Tell me what you want and let go of me” he tried to negotiate from his son.
The son was shocked for a while. He left the room, but returned 5 minutes later. “Dad”, he cried. “Can I have Rs 100/- from you please?”
On other times, Mr Iyer would have asked for reason, but this day was unlike that. He brought out his wallet and threw a Rs 100/- bill on his son. “Take this and leave” he demanded.
The boy picked up the cash from the floor and left. He returned in next five minutes. “Dad”, he said softly this time.
Mr Iyer’s temper was rising. He was getting annoyed. He grinded his teeth and asked in a stern voice, “What now?” “Please see” the son demanded.
Mr Iyer decided that unless some force is applied, this disturbance would persist. He got up from his chair and was about to thrash his son, when he noticed the clutched fist of his son held towards him.
“What is in your hand?” Mr Iyer asked angrily.
His son slowly unclenched his fist. There were few notes of various designations in the hand.
Mr Iyer was perplexed. “What are you doing? What do you want?” he asked in the same tone of annoyance.
“I opened my piggy bank. It had Rs 400/- I have taken Rs 100/- from you.”
“So?” the tone of Mr Iyer was mellowing down.
“I have heard you telling many uncles that you earn Rs 1000/- per hour.”
Mr Iyer was silent this time. His son continued.
“I have Rs 500/- with me. Can you please take this and play with me for half an hour?”
Mr Iyer went back to his laptop and shut it down. He played and spent time with his son the rest of the day. Mr Iyer was so much pleased with himself that his presentation the next day was applauded by everyone as the best he had done till then.
Very often, we do not understand the value small things. What we perceive as a small issue may really be a big one for the others. Our few minutes may be what the opposite person desperately needs. It is true with our family members and patients too. Often, big-wigs of the hospitals find it futile to find any time for their subordinates and patient attendants. I have seen busy practitioners starting their day around 5 pm and going home back by midnight. They are proud of their time management skills and claim that they stretch a couple of hours sleep in their cars during the travel. Few also say that their kids would not have woken up when they leave the house and would have slept well by the time they reach home. One of our consultants was very angry that he had to apply leave for a day to attend his son’s school day, as the boy was insisting on it a lot. It is very common in medical profession to have such scenarios. What majority does usually become a rule and makes an easy way others to follow. Doctors who try to defy the rule are termed “lazy”, “useless”, “waste body” and so on. It may be prudent to understand what the time management actually stands for before deciding on to invest on it. This small anecdote can open up lot of these issues pertinent to each of us in its own way.
With this, let us go back to the interesting learning scenarios of this post.
PROBLEM WITH WINDOWS
We had a situation which took lot of cerebral exercise. This 11-year-old boy had undergone closure of AP Window and PDA few years back. He came back to us with exertional dyspneoa. On echo, we reported him as possible residual PDA as suggested by the location of jet. However, on cath study, the outcome was a residual AP window. The data showed operability on oxygen study. During the cath meeting, our senior surgeon had a different opinion. He felt that the cause of this residual APW after these many years might be due to high PVRI per se and the residual opening is actually a pop-off. In his opinion, such lesions should be left alone if the symptoms are not much pressing. But the data we had did not suggest the same. Hence, we tried to attempt device closure of the lesion. We initially tried a VSD device, but it slipped. After few days, we tried a PDA device unsuccessfully. Now, we may not attempt the non-surgical ways anymore. What is the opinion of the readership on this? We shall keep you informed on the progress of this boy. Let me know your ideas on this issue.
PULMONARY CONTRIBUTION
We often see Qp/Qs in cath studies that are too high to believe. Yet, the pressure from dilated pulmonary arteries on the bronchi is not too high to compress the respiratory tract. We had a 8-month-old blue boy with large ASD shunting bidirectional and multiple muscular VSDs shunting right to left. The CHD lesions could not explain the clinical scenario. We found reduced air entry on the left lung. The chest radiograph showed collapsed left lung. The cardiac CT showed a completely collapsed left bronchus with collapsed left lung and a dilated left pulmonary artery adjacent to the bronchus. How to chronalize the cause-effect relationship? The surgical team felt that the dilated PA must have compressed the Left bronchus and the resultant lung collapse must have contributed to the PVRI causing right to left shunt. But, haven’t we seen ample number of large ASDs? How many times do we recall such a picture happening? Our logic was different. There might be an inherent defect in the left bronchus, which might have collapsed early due to added pressure by the LPA. Who is correct? We asked for pediatric surgical opinion for which a bronchoscopy for learning about the status of left bronchus. But the bronchoscopy was not much helpful. We were not clear on how to proceed. I hope the readership can also participate in this cerebral exercise for a while and come out with their suggestion.
BALLOON DECISIONS
How far is the balloon occlusion helpful in decision making of PDA operability? The technique or the interpretation does not seem to be standardized. We had an adult lady with a decent PDA shunting left to right. The balloon occlusion data was not helpful either way. We still went ahead with the device closure based on clinical and echo data. It turned out to be successful. The patient went home well. If we had gone by the cath data alone, we probably would have not touched the patient. Taking the entire picture, the cath data on balloon occlusion was invalid. Can the readership inform any published data on the proper balloon occlusion technique and interpretation? Please let me know.
TRACING THE PROBLEM
It was a nice question which we never had thought prior. The LV and RV tracings in the cath are very characteristic and reproducible. What causes the difference in the morphology of the tracing pattern? The question came up when one of the students put up this question in an e-class. “What does the LV tracing look like in a TGA?” Logical answer may be “like an RV tracing”. But, what is the correct answer? Please let me know the answer with references.
DISGUISING AS TAPVC
We had a 2-month-old who came to us with an echo report from outside as TAPVC. On the echo, we were surprised with the left to right shunting across the ASD. The left atrium looked very small with a normal mitral valve. The pulmonary veins were seen draining into the left atrium. Why was the LA small? On a close look, we found a bleak line within the presumed LA outside which no colour percolated. We decided that the echo free space is likely to be a cyst. Cardiac CT defined the mass to be cystic. On the surgical table, the cystic lesion was confirmed. There was no TAPVC. The smallish LA was secondary to an external compression by a cystic mass. It was only because of a machine with good resolution that we could pick up the diagnosis. I wonder what I could have done with a suboptimal machine that I am usually handed with at peripheral centres! If the readership has come across any similar events, please let us know.
PEDIATRIC CARDIOLOGY PEARLS:
11. The embrtological sequence of atrial septation is one of the most interesting understandings for both students and examiners. This hypothesis has been successful in explaining the abnormalities of atrial sepatations. This sequence of events was explained for the first time by van Mierop in 1976. Even today, we follow the same with few minor modifications. (Van Mierop LHS. In: Feldt RH, McGoon DC, Ongley PA, et al., eds. Atrioventricular Canal Defects. WB Saunders publications, 1976: page 12)
12. Children with Down syndrome are more likely to have complete AVSD than children without Down syndrome. They are also more likely to have associated tetralogy of Fallot (Vet TW, Ottenkamp. J in American Journal of Diseases in Children 1989, page 1362)
13. VSD happens to have most variants of classification. The most accepted version was published by Soto et al in 1980. In this classification, the ventricular septum is considered to have four components: An inlet septum separating the mitral and tricuspid valves; a trabecular septum, which extends from the attachments of the tricuspid leaflets outward to the apex and upward to the crista supraventricularis; the smooth-walled outlet or infundibular septum, which extends from the crista to the pulmonary valve; and the membranous septum, which is relatively small and is usually divided into two parts by the septal leaflet of the tricuspid valve. Each zone has defects going by their generic name. (Soto B, Becker AE, Moulaert AJ, et al, British Heart Journal 1980, page 33)
14. The incidence of PDA is about 30 times greater at high altitude (4,500 to 5,000 m) than at sea level (Alzamora-Castro V, Battilana G, Abugattas R, et al. American Journal of Cardiology 1960, page 761)
15. In Tetralogy of Fallot, about 40% of patients have an abnormally long, large conus artery from the right coronary artery that supplies a significant mass of myocardium. In 4% to 5%, the left anterior descending coronary artery arises from the right coronary artery and passes across the right ventricular outflow tract (RVOT). The resultant abnormal anatomy leads to compromises for surgical repair in the region of RVOT and pulmonary annulus. Hence, if the echo does not pick up the anomaly, one needs cath for proper surgical planning. (Fellows KE, Freed MD, Keane JF, et al. Circulation journal 1975, page 561)
With this, the present post ends. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am planning to add few more things to the new segment. Please ask your general pediatric friends about the blog and get their feedback on what would their need be.
Regards
Kiran
ಶನಿವಾರ, ಏಪ್ರಿಲ್ 17, 2010
Welcome to NH Blog. This is Dr Kiran. We have been exploring the wonderful world of pediatric cardiology for past few months. As previous, we shall continue with small anecdotes that convey more meanings.
The following story reached me via internet. One of the followers of this blog asked me to share this others. Due to credit to him, please go through this wonderful anecdote.
“How many times have you appreciated someone for no reason?” asked a renowned speaker in an annual meeting of a big company.
Eyebrows rose. “Why should we?” questioned the minds of top brass. “They have no eyes to appreciate the good, forget the same for no reason” mumbled the lower rung.
“Now think honestly for yourself; how many times you really felt like appreciating someone for their good work and did not do it for reasons that need not be disclosed?”
The hall went silent. The introspection was evident.
“After sometime”, continued the speaker, “it is very easy to forget the good job and the person who did it. Even if we try hard, we may not recall both the person and work. As the human nature goes, we forget the entire episode very easily.”
“So”, cried a voice from the middle of the crowd.
“Don’t postpone” said the wise man. “You have lost lot of opportunities to sincerely represent your feelings. Now, you can atone for it by appreciating someone who has done service to you. Let it be for no reason. Let it be a surprise for them. Let it come from nowhere for them. Try it out. Both of you will really feel better” he concluded.
The CEO of the firm thanked the speaker in his vote of thanks. "Our distinguished speaker made an exemplary point in his speech. We have been working on traditional moulds and outsets which preach that our relationships must be all business, cognitive, stand-offish and nonemotional. The most powerful thing he has taught us today is to inculcate a corporate culture with emotional human connection. From now on, I request all of you to freely express your appreciation to each other. This stuff could be contagious for a better world. Pass it on!"
When the function got over, the CEO was still haunted by the happenings of the day. On his way back home, he thanked his driver. "Thanks," he said. "For four years you have been my company driver and I don't think I have ever told you how wonderful you are. You are the best driver on the road. You are a first-class gentleman and an asset to our organization. I need you and deeply appreciate your friendship and loyalty."
The chauffeur was ecstatic. The CEO of the firm saying all the good things to him looked unbelievable. He was whistling and smiling like never before when he reached home. He went to his wife, hugged her and told, “I am feeling great today. I feel I have really achieved something. Lots of it is because of you. You are such a support and love. I don’t think I would have been the same without you. Thank you. Let’s go out for dinner tonight.”
The couple went to a nearby restaurant which was a tad expensive for them, but the man was too happy to afford that the day. Their son, who was sent out of the house for some reason a year back, worked there. The son didn't know what to say when he saw the parents. His father did something he could not believe. The father walked straight over to his son and hugged him. With tears in his eyes he said, "I love you, son. I appreciate you for what you are. Please come home. I miss you and need a relationship with you."
The boy went nervous. He had never seen his father like this. He excused himself from his parents and went into the manger’s chamber. "I've decided not to quit this job, sir. I have decide not to move out of this town," he told the manager. "I've just decided to move back in with my parents.” “I always thought your father was a strict disciplinarian. He never approved the free lifestyle you wanted to have. The boys tell me that you were sent out of the house when you got your body tattooed and changed your hair colour to join the music band. Why is the sudden change of decision?" the manager asked. "I just discovered my parents actually care about me and I'm sick of letting them down trying to prove my independence. It's time to make them proud!"
The story opens up a lot of things. There are lot of ways this can be interpreted. A genuine expression of appreciation is contagious and leads to good cascade. It is just the story of one person who followed it. The others who followed it might have had similar effects. The corporate culture has to break away from the mould. But, one word of warning. People with lesser maturity may not appreciate the difference between independence and indiscipline. So, even when a word of appreciation is handed over, the recipient should be really worth it. Try it out. It may be worth it.
With this, let us move on to our learning scenarios segment:
LOCATION: MATTERS?
Does the location of VSD matter for the velocity of the development of PVRI? In one of our meetings it was mentioned that between perimembranous VSD and subpulmonic VSD of same size, the latter develops early PVRI. I could not find any reference for the same. It can be logically concluded that the perimembranous VSDs have a chance of developing RVOT muscle bundles, but the subpulmonics would not. Also, the closer the VSD to the pulmonic valve, higher the chances of them behaving like aortopulmonary shunts. Does this theory find any takers? Are there any studies or observations to prove them? If similar findings are observed by you, please let me know.
JUXTAPOSED TGA
How common is it to find juxtaposed atrial appendage in TGA? We had a 2-month-old with TGA, intact IVS with juxtaposed atrial appendages. We had no recall of the last time we had this combination. Also, this baby had a smallish RPA with a normal sized LPA. How would one go about treating this combination? Our surgical team felt that a Senning would not be possible due to juxtaposition. The age and anatomy were not in favour of arterial switch. But, how to explain the disparity in the sizes of PAs? Has the appendage juxtaposition got anything to do with branch PA anatomy? Are they related in any way? Please let me know your ideas on it.
DISCONTINUITY
One of the major criteria for diagnosis of DORV is mitral-aortic discontinuity. When the great arteries are malposed, it would be mitral-pulmonic discontinuity. Does the same criteria hold good for DOLV too? Recently, we had a 6-month-old with this picture. AV concordance was clear. But, the PA was posterior and was completely committed to LV. There was a perimembranous malaligned VSD, with 25% override of d-posed anterior aorta. The mitral valve was clearly discontinuous with the posterior pulmonic valve. I reported it as TGA. However, my senior consultant changed the report to DORV based on the mitral-pulmonic discontinuity. Is it acceptable to call it a DORV when the posterior great vessel is totally committed to LV? Is the discontinuity clause a part of any Double outlet physiology or meant only for DORV? Since the number of DOLV are less, none of were very sure of this. I would like to know the opinion from the readership on this issue. If you come across such a picture, how would the report go? Please let me know.
TAPVC INTACT IAS
It was the second time in the recent past that we have come across a baby with TAPVC and intact IAS. The first one had a non-restrictive VSD and MR. The second one was latest – cardiac TAPVC with no detectable ASD or VSD and with only a restrictive PDA on echo. The surgical table found the final truth. There was indeed a small PFO, no VSD, restrictive PDA. But the surprise element was a partially unroofed coronary sinus, which went unnoticed on echo. It was very nice to know how the nature devises methods for the survival of the life. Any TAPVC with no flow across the MV is not known to survive, making the ASD near mandatory. Among the children of TAPVC with no ASD in our observation, the first one achieved a flow across the MV by MR and second one by a partially unroofed CS. It would be interesting to know the experiences of the readership. If you have come across any TAPVC with intact IAS, please let us know what other lesions led to a successful survival of the baby.
PRIMARY PROBLEM
Sometimes, certain situations can be perplexing with opinions on both poles. We had a 4-month-old with a large VSD shunting Right to Left, along with dilatation of RA and RV. There were no syndromic associations in this baby. It is very likely that the primary pulmonary resistance in this baby never dropped. It is extremely unlikely that the baby has already into Eisenmengarization. Now, in such scenarios, any further investigation may not yield useful information. How should this baby be approached? One end of spectrum is for a trial of sildenafil. On the other end lies suggestion for surgery. What way the readership vote? Is there a via-media way too? Please let me know your approach.
PEDIATRIC CARDIOLOGY PEARLS:
6. In a patient with typical auscultatory findings of an ASD and a P-wave axis of <30 degrees on the electrocardiogram, one should think immediately of a sinus venosus defect (Davia et al AHJ, 1973 page 180)
7. AV Canal defect is found commonly in children with Down syndrome. However, few specific characteristics are noted in Down syndrome:
• Situs and splenic anomalies are rare.
• They usually do not have associated LVOT obstruction, left ventricular hypoplasia, coarctation of the aorta, or additional muscular VSDs.
• Balanced AV canal defect is the commonest variant
(Marino. Cardiology in the Young, 1992, page 308)
8. Tachypneoa is a common symptom found in heart lesions with increased pulmonary blood flow (eg: ASD, VSD, PDA etc).
• In the absence of infection, the cardiovascular basis for the respiratory symptoms in such conditions probably is pulmonary edema of mild to moderate degree with elevated pulmonary venous pressure and decreased lung compliance.
• In infants with a large left-to-right shunt secondary to a VSD, dyspneoa occurs when the mean left atrial pressures reaches around 15 mm Hg.
(Donald. Progress in cardiovascular diseases, 1958, page 298)
9. In the fetus, the right ventricle ejects about two thirds of combined ventricular output, and because lung flow is only 6% to 8%, the ductus arteriosus carries 55% to 60% of combined ventricular output. (Heymann et al, Physiology review, 1975, page 65)
10. In any child with LV dysfunction, ECG is mandatory. If the ECG shows abnormal Q waves in leads I, aVL, and precordial leads V4 to V6 with or without abnormal R waves or R-wave progression in the left precordial leads, the likelihood of ALCAPA (Abnormal Left Coronary from Pulmonary Artery) is very high. All such children should undergo immediate Echocardiography in expert hands with an echo machine offering maximum resolution. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 708)
With this, we come to the end of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am yet to receive comments on improving the new segment. Please ask your general pediatric friends about the blog and get their feedback.
Regards
Kiran
The following story reached me via internet. One of the followers of this blog asked me to share this others. Due to credit to him, please go through this wonderful anecdote.
“How many times have you appreciated someone for no reason?” asked a renowned speaker in an annual meeting of a big company.
Eyebrows rose. “Why should we?” questioned the minds of top brass. “They have no eyes to appreciate the good, forget the same for no reason” mumbled the lower rung.
“Now think honestly for yourself; how many times you really felt like appreciating someone for their good work and did not do it for reasons that need not be disclosed?”
The hall went silent. The introspection was evident.
“After sometime”, continued the speaker, “it is very easy to forget the good job and the person who did it. Even if we try hard, we may not recall both the person and work. As the human nature goes, we forget the entire episode very easily.”
“So”, cried a voice from the middle of the crowd.
“Don’t postpone” said the wise man. “You have lost lot of opportunities to sincerely represent your feelings. Now, you can atone for it by appreciating someone who has done service to you. Let it be for no reason. Let it be a surprise for them. Let it come from nowhere for them. Try it out. Both of you will really feel better” he concluded.
The CEO of the firm thanked the speaker in his vote of thanks. "Our distinguished speaker made an exemplary point in his speech. We have been working on traditional moulds and outsets which preach that our relationships must be all business, cognitive, stand-offish and nonemotional. The most powerful thing he has taught us today is to inculcate a corporate culture with emotional human connection. From now on, I request all of you to freely express your appreciation to each other. This stuff could be contagious for a better world. Pass it on!"
When the function got over, the CEO was still haunted by the happenings of the day. On his way back home, he thanked his driver. "Thanks," he said. "For four years you have been my company driver and I don't think I have ever told you how wonderful you are. You are the best driver on the road. You are a first-class gentleman and an asset to our organization. I need you and deeply appreciate your friendship and loyalty."
The chauffeur was ecstatic. The CEO of the firm saying all the good things to him looked unbelievable. He was whistling and smiling like never before when he reached home. He went to his wife, hugged her and told, “I am feeling great today. I feel I have really achieved something. Lots of it is because of you. You are such a support and love. I don’t think I would have been the same without you. Thank you. Let’s go out for dinner tonight.”
The couple went to a nearby restaurant which was a tad expensive for them, but the man was too happy to afford that the day. Their son, who was sent out of the house for some reason a year back, worked there. The son didn't know what to say when he saw the parents. His father did something he could not believe. The father walked straight over to his son and hugged him. With tears in his eyes he said, "I love you, son. I appreciate you for what you are. Please come home. I miss you and need a relationship with you."
The boy went nervous. He had never seen his father like this. He excused himself from his parents and went into the manger’s chamber. "I've decided not to quit this job, sir. I have decide not to move out of this town," he told the manager. "I've just decided to move back in with my parents.” “I always thought your father was a strict disciplinarian. He never approved the free lifestyle you wanted to have. The boys tell me that you were sent out of the house when you got your body tattooed and changed your hair colour to join the music band. Why is the sudden change of decision?" the manager asked. "I just discovered my parents actually care about me and I'm sick of letting them down trying to prove my independence. It's time to make them proud!"
The story opens up a lot of things. There are lot of ways this can be interpreted. A genuine expression of appreciation is contagious and leads to good cascade. It is just the story of one person who followed it. The others who followed it might have had similar effects. The corporate culture has to break away from the mould. But, one word of warning. People with lesser maturity may not appreciate the difference between independence and indiscipline. So, even when a word of appreciation is handed over, the recipient should be really worth it. Try it out. It may be worth it.
With this, let us move on to our learning scenarios segment:
LOCATION: MATTERS?
Does the location of VSD matter for the velocity of the development of PVRI? In one of our meetings it was mentioned that between perimembranous VSD and subpulmonic VSD of same size, the latter develops early PVRI. I could not find any reference for the same. It can be logically concluded that the perimembranous VSDs have a chance of developing RVOT muscle bundles, but the subpulmonics would not. Also, the closer the VSD to the pulmonic valve, higher the chances of them behaving like aortopulmonary shunts. Does this theory find any takers? Are there any studies or observations to prove them? If similar findings are observed by you, please let me know.
JUXTAPOSED TGA
How common is it to find juxtaposed atrial appendage in TGA? We had a 2-month-old with TGA, intact IVS with juxtaposed atrial appendages. We had no recall of the last time we had this combination. Also, this baby had a smallish RPA with a normal sized LPA. How would one go about treating this combination? Our surgical team felt that a Senning would not be possible due to juxtaposition. The age and anatomy were not in favour of arterial switch. But, how to explain the disparity in the sizes of PAs? Has the appendage juxtaposition got anything to do with branch PA anatomy? Are they related in any way? Please let me know your ideas on it.
DISCONTINUITY
One of the major criteria for diagnosis of DORV is mitral-aortic discontinuity. When the great arteries are malposed, it would be mitral-pulmonic discontinuity. Does the same criteria hold good for DOLV too? Recently, we had a 6-month-old with this picture. AV concordance was clear. But, the PA was posterior and was completely committed to LV. There was a perimembranous malaligned VSD, with 25% override of d-posed anterior aorta. The mitral valve was clearly discontinuous with the posterior pulmonic valve. I reported it as TGA. However, my senior consultant changed the report to DORV based on the mitral-pulmonic discontinuity. Is it acceptable to call it a DORV when the posterior great vessel is totally committed to LV? Is the discontinuity clause a part of any Double outlet physiology or meant only for DORV? Since the number of DOLV are less, none of were very sure of this. I would like to know the opinion from the readership on this issue. If you come across such a picture, how would the report go? Please let me know.
TAPVC INTACT IAS
It was the second time in the recent past that we have come across a baby with TAPVC and intact IAS. The first one had a non-restrictive VSD and MR. The second one was latest – cardiac TAPVC with no detectable ASD or VSD and with only a restrictive PDA on echo. The surgical table found the final truth. There was indeed a small PFO, no VSD, restrictive PDA. But the surprise element was a partially unroofed coronary sinus, which went unnoticed on echo. It was very nice to know how the nature devises methods for the survival of the life. Any TAPVC with no flow across the MV is not known to survive, making the ASD near mandatory. Among the children of TAPVC with no ASD in our observation, the first one achieved a flow across the MV by MR and second one by a partially unroofed CS. It would be interesting to know the experiences of the readership. If you have come across any TAPVC with intact IAS, please let us know what other lesions led to a successful survival of the baby.
PRIMARY PROBLEM
Sometimes, certain situations can be perplexing with opinions on both poles. We had a 4-month-old with a large VSD shunting Right to Left, along with dilatation of RA and RV. There were no syndromic associations in this baby. It is very likely that the primary pulmonary resistance in this baby never dropped. It is extremely unlikely that the baby has already into Eisenmengarization. Now, in such scenarios, any further investigation may not yield useful information. How should this baby be approached? One end of spectrum is for a trial of sildenafil. On the other end lies suggestion for surgery. What way the readership vote? Is there a via-media way too? Please let me know your approach.
PEDIATRIC CARDIOLOGY PEARLS:
6. In a patient with typical auscultatory findings of an ASD and a P-wave axis of <30 degrees on the electrocardiogram, one should think immediately of a sinus venosus defect (Davia et al AHJ, 1973 page 180)
7. AV Canal defect is found commonly in children with Down syndrome. However, few specific characteristics are noted in Down syndrome:
• Situs and splenic anomalies are rare.
• They usually do not have associated LVOT obstruction, left ventricular hypoplasia, coarctation of the aorta, or additional muscular VSDs.
• Balanced AV canal defect is the commonest variant
(Marino. Cardiology in the Young, 1992, page 308)
8. Tachypneoa is a common symptom found in heart lesions with increased pulmonary blood flow (eg: ASD, VSD, PDA etc).
• In the absence of infection, the cardiovascular basis for the respiratory symptoms in such conditions probably is pulmonary edema of mild to moderate degree with elevated pulmonary venous pressure and decreased lung compliance.
• In infants with a large left-to-right shunt secondary to a VSD, dyspneoa occurs when the mean left atrial pressures reaches around 15 mm Hg.
(Donald. Progress in cardiovascular diseases, 1958, page 298)
9. In the fetus, the right ventricle ejects about two thirds of combined ventricular output, and because lung flow is only 6% to 8%, the ductus arteriosus carries 55% to 60% of combined ventricular output. (Heymann et al, Physiology review, 1975, page 65)
10. In any child with LV dysfunction, ECG is mandatory. If the ECG shows abnormal Q waves in leads I, aVL, and precordial leads V4 to V6 with or without abnormal R waves or R-wave progression in the left precordial leads, the likelihood of ALCAPA (Abnormal Left Coronary from Pulmonary Artery) is very high. All such children should undergo immediate Echocardiography in expert hands with an echo machine offering maximum resolution. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 708)
With this, we come to the end of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. I am yet to receive comments on improving the new segment. Please ask your general pediatric friends about the blog and get their feedback.
Regards
Kiran
ಮಂಗಳವಾರ, ಏಪ್ರಿಲ್ 13, 2010
Welcome to NH Blog. This is Dr Kiran anchoring you to this journey. We shall continue with anecdotes that convey more meanings.
I recalled this story when my erstwhile boss was narrating her experience in one of the hospital as attendant of a patient.
Divya was a cherubic, bubbly girl of 4 years. She started going to a nearby school. She was supposed to come back from the school with another baby from the neighbourhood along with an attendant. One day, the attendant of the neighbour boy told Divya’s mother that Divya refused to come with him and she is staying back in the school.
Her mom was surprised. Although Divya was naughty girl, she never did anything similar before in the past 4 months of school. A bit tensed, her mom walked towards the school.
At a small bench near the play area, Divya was sitting alone. Her face was not its usual bright.
“What happened to you?” demanded her mom.
“Nothing, mom”, replied Divya, still with some gloom on her face.
“Did anyone say anything to you?”
“Nope!”
“Then why are you not home yet? Why did you refuse to come with that uncle?” her mother sounded angry now.
“Actually, mom” Divya started. “My friend, you know, dropped her doll and it broke”
“So?”
“I was with her.”
“Did you stay back to repair her broken doll?” ridiculed her mother in a taunting way.
“Oh! mom. I don’t know how to repair her doll. I just stayed back when she was crying”
“Which friend? What is her name? Where is she now?
“I don’t know her name. She is my friend. She left just now with her dad. I saw you coming and stayed back.”
“What did you say when she was crying?” asked her mom, getting more curious now.
“I did not say anything. I just cried with her”
Her mom was speechless with overwhelming emotions.
It is quite satisfying to have a person standing by you silently in times of agony. It is probably one place where the hospital management has to look at. We usually have indifferent staff, busy nurses, super-busy doctors and all the other people in the hospital. Management is usually engaged in the balance sheets. Hardly anyone thinks of the situation from the point of patient’s family. Should we really face the situation head-on as a patient before realising the feelings they go through? That is too expensive way of learning things. Can we be more sympathetic for situations which demand common emotions from the entire humankind? The little girl in the above anecdote is probably teaching us a superior method of living as humans.
Let us get back to the section of interesting learning scenarios once again:
TOF with TAPVC
We recently had a couple of children with abnormal pulmonary venous connections associated with tetralogy of Fallot. First baby which was 1-year-old had two pulmonary veins entering the RA and the other 2 entering LA. This had gone to OT table, unnoticed in the echo. Second one was a much higher shocker! This 4-year-old had very poor echo windows and cath study was suggested to evaluate for additional VSDs. In the cath, the RA catheter entered RUPV. Hand injection of contrast showed the dye entering RA. The surgeon was informed about the association of PAPVC. However, on the table, the surprise was revealed. The baby had TAPVC! Was the hand injection of dye in cath not enough to opacify common confluence? We went back to the echo recordings, but the windows were so poor that we could not get any information on pulmonary venous return, especially in this baby with very small Qp. I am seriously considering a prospective study on the differences found on the OT table against the final diagnosis provided by the cardiology team. How are the experiences of other centres? Please let us know your experiences.
CHANGING PATH(OE)S!
We came across a 3-year-old operated for Tetralogy 2 years back. The boy was followed up in his native and came to us for the first time after the surgery. He was otherwise doing well, with no symptoms. On echo, we could not find LPA either in 2D or colour in any of the conventional views. The flow across RPA appeared normal. However, on clinical examination, his left lung had normal breath sounds, no added sounds. We wondered why the LPA was not visible. Small clue on chest radiograph was that the Left dome of diaphragm was high up with normal vascular pattern on both lung fields. It was probably paralysed during the surgical procedure and must be partially recovering now. It was likely that the orientation of lung has changed in such a way that the LPA must be traversing in a very odd angle and limited the visibility of LPA. This was only a hypothesis and we could not find any better explanation. We did not get any further investigations, as the clinical picture was very satisfactory and virtually no clues of abnormality in physical examination. Any experiences with such a pattern? If anyone has any better explanations, I would like to hear. How do you think such kids should be investigated? Please let me know your opinions on it.
NO COMPACT OPINION
Many times, we come across number of children with ventricles looking similar to non-compaction. What are the actual criteria for this diagnosis? Is a simple visual impression enough to say this? Are there any clear cut guidelines to say so? The text books describe it as spongy appearance of ventricular surface with thin epicardial layer and a thickened endocardial layer with prominent trabeculations and deep recesses. How to objectively evaluate this entity, which has only visual description? How many times do we really see ventricular dysfunction associated with this picture? How are the experiences with other centres? Please let me know your take on it.
DOUBLE DYSPLASIA
It was a child which disturbed our team a lot. This 3-month-old boy had truncus arteriosus. However, the truncal valve was quadricuspid and dysplastic. There was no other reason why this baby should not have had further procedures. However, is it worthwhile to convert this leaking dysplastic valve (which is not even repairable) into the future aortic valve? Is there any other option than rejecting the scenario as “untreatable”? Has anyone come across a similar phenomenon earlier? Please let me know your views on this.
REGURGITATIONS IN OPERABILITY
On an interesting note, we had three children with AP Window on a single day in our OPD. It was more than a coincidence. One among them was a 9-month-old with type 1 APW. This child also had mitral and tricuspid valve prolapses with severe regurgitations along with moderate aortic regurgitation. The net result was dilatation of all the cardiac chambers with reversal of flow in the aortic arch. The pulmonary venous return on the echo could not be assessed satisfactorily. Now the question was: How would one assess the operability on the echo for such children? The child saturated 93% in room air. Is that enough to claim operability? Is the cath study mandatory? Let me know how you would handle this issue.
Some of my friends who are pediatricians with lots of academic interest had an objection about the blog. They claim that it is too much of pediatric cardiology alone. They wanted something that would interest the general pediatric person with interest in Pediatric cardiology. Also, the post-graduate students in pediatrics wanted something that would interest them. With these in mind, I have decided to start a new section from now on. This section would be called “Pediatric cardiology pearls”. I would give 5 interesting and practical points in pediatric cardiology from a known work, along with the source. It should be good for the practitioner and students for management and quoting in the scientific meetings as well. Here goes the first instalment:
PEDIATRIC CARDIOLOGY PEARLS:
1. In the ECG of ASD, one can often see a notch near the apex of R wave in leads 2, 3 and avF which resembles the croche needle. This is called the crochetage sign. This has high specificity for the diagnosis of ASD if found along with the rsR’ pattern in the ECG. Contrary to the popular belief, the crochetage sign does not have a steady correlation with the shunt severity and is often found with PFO also. (Heller et al in JACC 1996, page 877)
2. An Eisenmenger VSD (also called Eisenmenger malalignment) is a perimembranous VSD with an anteriror deviation of infundibular septum. This has nothing to do with Eisenmenger syndrome or Eisenmenger complex (shunt reversal due to high pulmonary vascular resistance). (Fukuda et al in Cardiology in the Young 2000, page 343)
3. PDA is classified into 5 types: wide aortic end, wide pulmonary end, tubular, multiple constrictions and bizarre. (Krichenko et al AJC, 1989, page 879)
4. In the AV canal defect, distance from the cardiac crux to the left ventricular apex is foreshortened, and the distance from the apex to the aortic valve is increased. As a result, the LVOT is longer and narrower than normal and produces the “gooseneck” deformity. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 646)
5. A combination of persistent left superior vena cava terminating in left atrium, atrial septal defect, and absence of roof of coronary sinus is called Raghib complex. It is associated with cyanosis – one of the few causes for cyanosis in ASD without Eisenmengarization (Raghib et al Circulation 1965, page 912)
With this, we come to the conclusion of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. Thanks for all the new followers. Please let me know how the new segment can be improved with the defined objectives that I have mentioned.
Regards
Kiran
I recalled this story when my erstwhile boss was narrating her experience in one of the hospital as attendant of a patient.
Divya was a cherubic, bubbly girl of 4 years. She started going to a nearby school. She was supposed to come back from the school with another baby from the neighbourhood along with an attendant. One day, the attendant of the neighbour boy told Divya’s mother that Divya refused to come with him and she is staying back in the school.
Her mom was surprised. Although Divya was naughty girl, she never did anything similar before in the past 4 months of school. A bit tensed, her mom walked towards the school.
At a small bench near the play area, Divya was sitting alone. Her face was not its usual bright.
“What happened to you?” demanded her mom.
“Nothing, mom”, replied Divya, still with some gloom on her face.
“Did anyone say anything to you?”
“Nope!”
“Then why are you not home yet? Why did you refuse to come with that uncle?” her mother sounded angry now.
“Actually, mom” Divya started. “My friend, you know, dropped her doll and it broke”
“So?”
“I was with her.”
“Did you stay back to repair her broken doll?” ridiculed her mother in a taunting way.
“Oh! mom. I don’t know how to repair her doll. I just stayed back when she was crying”
“Which friend? What is her name? Where is she now?
“I don’t know her name. She is my friend. She left just now with her dad. I saw you coming and stayed back.”
“What did you say when she was crying?” asked her mom, getting more curious now.
“I did not say anything. I just cried with her”
Her mom was speechless with overwhelming emotions.
It is quite satisfying to have a person standing by you silently in times of agony. It is probably one place where the hospital management has to look at. We usually have indifferent staff, busy nurses, super-busy doctors and all the other people in the hospital. Management is usually engaged in the balance sheets. Hardly anyone thinks of the situation from the point of patient’s family. Should we really face the situation head-on as a patient before realising the feelings they go through? That is too expensive way of learning things. Can we be more sympathetic for situations which demand common emotions from the entire humankind? The little girl in the above anecdote is probably teaching us a superior method of living as humans.
Let us get back to the section of interesting learning scenarios once again:
TOF with TAPVC
We recently had a couple of children with abnormal pulmonary venous connections associated with tetralogy of Fallot. First baby which was 1-year-old had two pulmonary veins entering the RA and the other 2 entering LA. This had gone to OT table, unnoticed in the echo. Second one was a much higher shocker! This 4-year-old had very poor echo windows and cath study was suggested to evaluate for additional VSDs. In the cath, the RA catheter entered RUPV. Hand injection of contrast showed the dye entering RA. The surgeon was informed about the association of PAPVC. However, on the table, the surprise was revealed. The baby had TAPVC! Was the hand injection of dye in cath not enough to opacify common confluence? We went back to the echo recordings, but the windows were so poor that we could not get any information on pulmonary venous return, especially in this baby with very small Qp. I am seriously considering a prospective study on the differences found on the OT table against the final diagnosis provided by the cardiology team. How are the experiences of other centres? Please let us know your experiences.
CHANGING PATH(OE)S!
We came across a 3-year-old operated for Tetralogy 2 years back. The boy was followed up in his native and came to us for the first time after the surgery. He was otherwise doing well, with no symptoms. On echo, we could not find LPA either in 2D or colour in any of the conventional views. The flow across RPA appeared normal. However, on clinical examination, his left lung had normal breath sounds, no added sounds. We wondered why the LPA was not visible. Small clue on chest radiograph was that the Left dome of diaphragm was high up with normal vascular pattern on both lung fields. It was probably paralysed during the surgical procedure and must be partially recovering now. It was likely that the orientation of lung has changed in such a way that the LPA must be traversing in a very odd angle and limited the visibility of LPA. This was only a hypothesis and we could not find any better explanation. We did not get any further investigations, as the clinical picture was very satisfactory and virtually no clues of abnormality in physical examination. Any experiences with such a pattern? If anyone has any better explanations, I would like to hear. How do you think such kids should be investigated? Please let me know your opinions on it.
NO COMPACT OPINION
Many times, we come across number of children with ventricles looking similar to non-compaction. What are the actual criteria for this diagnosis? Is a simple visual impression enough to say this? Are there any clear cut guidelines to say so? The text books describe it as spongy appearance of ventricular surface with thin epicardial layer and a thickened endocardial layer with prominent trabeculations and deep recesses. How to objectively evaluate this entity, which has only visual description? How many times do we really see ventricular dysfunction associated with this picture? How are the experiences with other centres? Please let me know your take on it.
DOUBLE DYSPLASIA
It was a child which disturbed our team a lot. This 3-month-old boy had truncus arteriosus. However, the truncal valve was quadricuspid and dysplastic. There was no other reason why this baby should not have had further procedures. However, is it worthwhile to convert this leaking dysplastic valve (which is not even repairable) into the future aortic valve? Is there any other option than rejecting the scenario as “untreatable”? Has anyone come across a similar phenomenon earlier? Please let me know your views on this.
REGURGITATIONS IN OPERABILITY
On an interesting note, we had three children with AP Window on a single day in our OPD. It was more than a coincidence. One among them was a 9-month-old with type 1 APW. This child also had mitral and tricuspid valve prolapses with severe regurgitations along with moderate aortic regurgitation. The net result was dilatation of all the cardiac chambers with reversal of flow in the aortic arch. The pulmonary venous return on the echo could not be assessed satisfactorily. Now the question was: How would one assess the operability on the echo for such children? The child saturated 93% in room air. Is that enough to claim operability? Is the cath study mandatory? Let me know how you would handle this issue.
Some of my friends who are pediatricians with lots of academic interest had an objection about the blog. They claim that it is too much of pediatric cardiology alone. They wanted something that would interest the general pediatric person with interest in Pediatric cardiology. Also, the post-graduate students in pediatrics wanted something that would interest them. With these in mind, I have decided to start a new section from now on. This section would be called “Pediatric cardiology pearls”. I would give 5 interesting and practical points in pediatric cardiology from a known work, along with the source. It should be good for the practitioner and students for management and quoting in the scientific meetings as well. Here goes the first instalment:
PEDIATRIC CARDIOLOGY PEARLS:
1. In the ECG of ASD, one can often see a notch near the apex of R wave in leads 2, 3 and avF which resembles the croche needle. This is called the crochetage sign. This has high specificity for the diagnosis of ASD if found along with the rsR’ pattern in the ECG. Contrary to the popular belief, the crochetage sign does not have a steady correlation with the shunt severity and is often found with PFO also. (Heller et al in JACC 1996, page 877)
2. An Eisenmenger VSD (also called Eisenmenger malalignment) is a perimembranous VSD with an anteriror deviation of infundibular septum. This has nothing to do with Eisenmenger syndrome or Eisenmenger complex (shunt reversal due to high pulmonary vascular resistance). (Fukuda et al in Cardiology in the Young 2000, page 343)
3. PDA is classified into 5 types: wide aortic end, wide pulmonary end, tubular, multiple constrictions and bizarre. (Krichenko et al AJC, 1989, page 879)
4. In the AV canal defect, distance from the cardiac crux to the left ventricular apex is foreshortened, and the distance from the apex to the aortic valve is increased. As a result, the LVOT is longer and narrower than normal and produces the “gooseneck” deformity. (Moss and Adams Pediatric cardiology text book, 7th Ed, page 646)
5. A combination of persistent left superior vena cava terminating in left atrium, atrial septal defect, and absence of roof of coronary sinus is called Raghib complex. It is associated with cyanosis – one of the few causes for cyanosis in ASD without Eisenmengarization (Raghib et al Circulation 1965, page 912)
With this, we come to the conclusion of another post. Please mail your suggestions and feedback to drkiranvs@gmail.com or use the comments section. Thanks for all the new followers. Please let me know how the new segment can be improved with the defined objectives that I have mentioned.
Regards
Kiran
ಇದಕ್ಕೆ ಸಬ್ಸ್ಕ್ರೈಬ್ ಆಗಿ:
ಪೋಸ್ಟ್ಗಳು (Atom)