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
ಶನಿವಾರ, ಮೇ 15, 2010
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
ಗುರುವಾರ, ಮಾರ್ಚ್ 11, 2010
Welcome back to NH blog. This is Dr Kiran. We are in the process of finding something pertinent to our medical lives hidden in some of the known anecdotes.
The following anecdote was quoted by one of my friends. He had read it in a magazine few years back. He found the end very touching and narrated to me.
The story goes like this:
Mr and Mrs Shaw were worried. Unlike other members of their family, their youngest son Rahul was not an academic achiever, despite reaching his fifth standard.
The parents gave him everything a child could desire. Their only motive was to make him an academic success. They wanted their son to top the class, as did their other children. They were telling Rahul the virtues of good academic career. They would quote how his elder siblings are respected in the family for their academic excellence and so on. Not that Rahul was stupid. He was naughty, mischievous, playful, spoke a lot of nonsense, played well, but could not study and get good grades. His teachers were fed up with him. They would often call the parents to school to report him. They advised the parents to seek the help of a counsellor to rule out attention deficit hyperactivity. Many teachers could not just bear his presence in their class. All these were reflected in his report card as low grades. The school authorities threatened the parents of a transfer certificate if they did not get any better of him.
And, suddenly Rahul became better. His grades started improving. His attention span increased. There were literally no complaints from the school for more than a fortnight. It all sounded and felt magical for the parents. They had not done anything from their end, but still, something seemed to work.
This time when the parents were called to the school, they expected another taunt, but were taken by a surprise. The teacher had called them to congratulate on their effort. Rahul had got good grades in the unit test. The parents could not digest this. They started feeling guilty for the appreciation bestowed on them for no effort of theirs.
But the event deserved celebration. Both parents took Rahul for an ice-cream. In the parlour, Mr Shaw asked his son: “How are you doing all this?” He was worried if his son had started copying from his neighbour.
“I did not see anyone’s paper. It is all my own effort” said the lad.
“But how?” demanded Mrs Shaw this time.
“I have got a new friend. She works in my school. I talk to her all the time. I feel great with it”
“In the school?” thought Mr Shaw. “If the effort is done by the school to improve him, why are they complimenting us?”
“Something’s not fitting” he told his wife. “Can you show me your new friend?” he asked his son.
“Of course. She would still be at school. We can go now and meet her” said Rahul with a happy note in the voice.
They turned their car towards the school. Rahul led them towards the library. They found a lady in her twenties, arranging the books in the racks.
“What is her name?” asked Mr Shaw.
“I don’t know” was the prompt response from the son!
They waited till the lady was done with her work and returned to the counter. She saw Rahul and looked happy.
“These are my parents” introduced Rahul.
The lady bowed to them gracefully and smiled.
“Thanks for helping my son. We don’t know how to thank you enough”, said Mrs Shaw gently.
The lady did not say anything. She showed few gestures with her hands, which neither of the Shaws understood. All that they could make out was that she was using sign language used by verbally handicapped.
“Oh my God! She cannot speak?” said a shocked Mrs Shaw.
“Not just that. She can’t hear, read or write either. She is employed by the school to keep the library clean” said the Library in-charge who was passing by.
The parents looked perplexed. Mr Shaw came out of the shock and demanded with his son: “What sort of help have you got from her that improved you so much? She can’t teach you any academics; she doesn’t know how to write. Forget other things – she can’t even hear you.”
Smile could not leave Rahul’s face. “She does far more than hearing, Dad. She LISTENS!!”
We often miss this point. Most of the times, we medical professionals hear or preach. We do not listen. I have a nice recall from my medical school times. One doctor who was considered just mediocre for his medical knowledge was very popular with patients. Similarly, another doctor we used to adore for the clinical acumen and great subject knowledge was a total flop with patients. When this was discussed amongst friends, we would flatly declare that the patients are foolish; they are so ignorant and dumb that they can’t even recognise a good doctor from a bad one. We felt that we knew the definition of “good” far better than the patient.
On the hind sight, now I feel that the patients were not outright foolish. They sought what they wanted and got it. The doctor who was mediocre in our view was good for them, because he LISTENED. Our “great man” either heard or preached. We often witness how emotional catharsis helps the patients in psychiatry. But, we fail to extrapolate the same for ourselves. Many a times, the patients need a soothing soul to pour out their inner self. A good listener. But how many of us can afford to do so in our busy practise? When time becomes money for the professional, where is the scope to be a good listener? But, there should be a way out. There are many doctors who have successfully balanced both very nicely during their entire career. If you ARE one or know one, please communicate with our readership. Let the world “LISTEN” to you this time.
With this, let’s get back to our regular feature: Challenges in Pediatric Cardiology!
VIAGRA CONTROL
It has been few years that we are using sildenafil for the pulmonary hypertension and high PVRI. Indiscriminate use is one of the biggest challenges bothering the congenital heart care. We have seen doctors equating PAH with sildenafil. Despite all the efforts of journals and experts trying to create awareness on judicious use of sildenafil, the misuse is rampant. Is there is any role of Bosentan? How are comparative analogies between Bosentan and Sildenafil? Please let us know if any studies on this comparison are published.
LIMITATIONS OF VALVE
We had a 32-year-old with small subpulmonic VSD, bicuspid aortic valve and moderate AR. The issue was with the valve. No doubt that the VSD needs closure as per guidelines. But, is the valve repairable? If yes, then the effort is worth it. If not, then the patient needs valve replacement. Is the valve replacement worth in a 32-year-old, especially when the hemodynamic effect of VSD is small? What happens if the lesion is left untouched now? Is it possible to close VSD alone and not attempt the valve repair at all? All these questions bothering us, the patient data was placed in front of the surgeons. The surgical team decided to go ahead with the VSD closure and assured that the valve can be repaired. Their analogy was simple. If the VSD is closed with the AR left alone, the natural history of bicuspid aortic valve would be far superior to that with the VSD pulling the aortic valve cusp. If VSD is not tackled now, the patient may deteriorate faster. What is the experience with other centres? How would you tackle this scenario? Please let me know.
CONVENTIONAL CONFUSION
Sometimes, we wonder how fast we forget! Once we reach a level of practical comfort, the theory that was read becomes easily effaced. Same happened during one of our seminars recently. All of us remembered the classification of Tricuspid atresia as I, II, III based on great artery relations and A, B, C based on the pulmonary valve and pressures. Although I am quite fond of history, I had forgotten that the mentioned classification goes with the eponym of Kuhne. Our fellow, Dr Shweta, was presenting the seminar. She told us that the class IA was intact IVS AND pulmonary atresia, class IB was restrictive VSD AND pulmonary valvar stensosis. I wondered if the term AND is correct. The feeling was that AND should be replaced by OR/AND. However, she showed the reference from the text book (Moss and Adams). I was unable to get the original article by Kuhne et al. If anyone has any recall or has the original article, I request you to pass it on for the benefit of others.
LOCATION, LOCATION, LOCATION
I am yet to find a person who has completely understood the VSDs! This simple looking lesion can pose so many surprises, that the moment you get comfortable with it, a new problem, hitherto not experienced, pops up. In one of our discussions, our senior surgeon opined that the outlet VSDs have development of early PVRI compared to VSDs in other locations. The opinion was seconded by others. I could not recall any statement of this sort in the texts. I presumed the probable reason to be the proximity with pulmonic valve. It is likely that the hemophysiology of outlet VSDs simulate AP Windows to a certain extent. Is my presumption correct? If anyone can come with proper explanation with proof, you will be rewarded by a special mention in the blog; your name would be written in CAPITAL LETTERS!!
BLUE OR NOT
We were coursing through some of the interesting cases seen off late. We do have a series of single pulmonary arteries unpublished yet. One of such cases was a hemitruncus with intact septae. The question was: would this patient ever by cyanosed? If yes, why and how? I was emphatic that such patients would never have a cardiac cyanosis. The pulmonary artery exposed to the high pressure would eventually develop high PVRI and restricts the blood flow. The entire right heart output is going to single lung and it may eventually develop high PVRI. Till then, the patient should be pink. After bilateral high PVRI, the patient may have signs of low cardiac output and failure rather than cyanosis, unless the PFO opens up or pulmonary AV fistulae develop. Hence, clinically, the loudness of S2 would determine the status of the patient. Soft S2 shows patient has at least one good lung and loud P2 shows the development of high PVRI in the hitherto good lung. However, there were some arguments against my logic. What is your opinion? We may not have seen such a scenario, but may logically conclude the outcome. Let me know your takes on this issue.
This scenario marks the end of another post. Let me get the suggestions and answers to all these queries. Put them in the comments box or mail them to drkiranvs@gmail.com Let’s see how best this platform can be utilised for the purpose of mutual learning.
Regards
Kiran
The following anecdote was quoted by one of my friends. He had read it in a magazine few years back. He found the end very touching and narrated to me.
The story goes like this:
Mr and Mrs Shaw were worried. Unlike other members of their family, their youngest son Rahul was not an academic achiever, despite reaching his fifth standard.
The parents gave him everything a child could desire. Their only motive was to make him an academic success. They wanted their son to top the class, as did their other children. They were telling Rahul the virtues of good academic career. They would quote how his elder siblings are respected in the family for their academic excellence and so on. Not that Rahul was stupid. He was naughty, mischievous, playful, spoke a lot of nonsense, played well, but could not study and get good grades. His teachers were fed up with him. They would often call the parents to school to report him. They advised the parents to seek the help of a counsellor to rule out attention deficit hyperactivity. Many teachers could not just bear his presence in their class. All these were reflected in his report card as low grades. The school authorities threatened the parents of a transfer certificate if they did not get any better of him.
And, suddenly Rahul became better. His grades started improving. His attention span increased. There were literally no complaints from the school for more than a fortnight. It all sounded and felt magical for the parents. They had not done anything from their end, but still, something seemed to work.
This time when the parents were called to the school, they expected another taunt, but were taken by a surprise. The teacher had called them to congratulate on their effort. Rahul had got good grades in the unit test. The parents could not digest this. They started feeling guilty for the appreciation bestowed on them for no effort of theirs.
But the event deserved celebration. Both parents took Rahul for an ice-cream. In the parlour, Mr Shaw asked his son: “How are you doing all this?” He was worried if his son had started copying from his neighbour.
“I did not see anyone’s paper. It is all my own effort” said the lad.
“But how?” demanded Mrs Shaw this time.
“I have got a new friend. She works in my school. I talk to her all the time. I feel great with it”
“In the school?” thought Mr Shaw. “If the effort is done by the school to improve him, why are they complimenting us?”
“Something’s not fitting” he told his wife. “Can you show me your new friend?” he asked his son.
“Of course. She would still be at school. We can go now and meet her” said Rahul with a happy note in the voice.
They turned their car towards the school. Rahul led them towards the library. They found a lady in her twenties, arranging the books in the racks.
“What is her name?” asked Mr Shaw.
“I don’t know” was the prompt response from the son!
They waited till the lady was done with her work and returned to the counter. She saw Rahul and looked happy.
“These are my parents” introduced Rahul.
The lady bowed to them gracefully and smiled.
“Thanks for helping my son. We don’t know how to thank you enough”, said Mrs Shaw gently.
The lady did not say anything. She showed few gestures with her hands, which neither of the Shaws understood. All that they could make out was that she was using sign language used by verbally handicapped.
“Oh my God! She cannot speak?” said a shocked Mrs Shaw.
“Not just that. She can’t hear, read or write either. She is employed by the school to keep the library clean” said the Library in-charge who was passing by.
The parents looked perplexed. Mr Shaw came out of the shock and demanded with his son: “What sort of help have you got from her that improved you so much? She can’t teach you any academics; she doesn’t know how to write. Forget other things – she can’t even hear you.”
Smile could not leave Rahul’s face. “She does far more than hearing, Dad. She LISTENS!!”
We often miss this point. Most of the times, we medical professionals hear or preach. We do not listen. I have a nice recall from my medical school times. One doctor who was considered just mediocre for his medical knowledge was very popular with patients. Similarly, another doctor we used to adore for the clinical acumen and great subject knowledge was a total flop with patients. When this was discussed amongst friends, we would flatly declare that the patients are foolish; they are so ignorant and dumb that they can’t even recognise a good doctor from a bad one. We felt that we knew the definition of “good” far better than the patient.
On the hind sight, now I feel that the patients were not outright foolish. They sought what they wanted and got it. The doctor who was mediocre in our view was good for them, because he LISTENED. Our “great man” either heard or preached. We often witness how emotional catharsis helps the patients in psychiatry. But, we fail to extrapolate the same for ourselves. Many a times, the patients need a soothing soul to pour out their inner self. A good listener. But how many of us can afford to do so in our busy practise? When time becomes money for the professional, where is the scope to be a good listener? But, there should be a way out. There are many doctors who have successfully balanced both very nicely during their entire career. If you ARE one or know one, please communicate with our readership. Let the world “LISTEN” to you this time.
With this, let’s get back to our regular feature: Challenges in Pediatric Cardiology!
VIAGRA CONTROL
It has been few years that we are using sildenafil for the pulmonary hypertension and high PVRI. Indiscriminate use is one of the biggest challenges bothering the congenital heart care. We have seen doctors equating PAH with sildenafil. Despite all the efforts of journals and experts trying to create awareness on judicious use of sildenafil, the misuse is rampant. Is there is any role of Bosentan? How are comparative analogies between Bosentan and Sildenafil? Please let us know if any studies on this comparison are published.
LIMITATIONS OF VALVE
We had a 32-year-old with small subpulmonic VSD, bicuspid aortic valve and moderate AR. The issue was with the valve. No doubt that the VSD needs closure as per guidelines. But, is the valve repairable? If yes, then the effort is worth it. If not, then the patient needs valve replacement. Is the valve replacement worth in a 32-year-old, especially when the hemodynamic effect of VSD is small? What happens if the lesion is left untouched now? Is it possible to close VSD alone and not attempt the valve repair at all? All these questions bothering us, the patient data was placed in front of the surgeons. The surgical team decided to go ahead with the VSD closure and assured that the valve can be repaired. Their analogy was simple. If the VSD is closed with the AR left alone, the natural history of bicuspid aortic valve would be far superior to that with the VSD pulling the aortic valve cusp. If VSD is not tackled now, the patient may deteriorate faster. What is the experience with other centres? How would you tackle this scenario? Please let me know.
CONVENTIONAL CONFUSION
Sometimes, we wonder how fast we forget! Once we reach a level of practical comfort, the theory that was read becomes easily effaced. Same happened during one of our seminars recently. All of us remembered the classification of Tricuspid atresia as I, II, III based on great artery relations and A, B, C based on the pulmonary valve and pressures. Although I am quite fond of history, I had forgotten that the mentioned classification goes with the eponym of Kuhne. Our fellow, Dr Shweta, was presenting the seminar. She told us that the class IA was intact IVS AND pulmonary atresia, class IB was restrictive VSD AND pulmonary valvar stensosis. I wondered if the term AND is correct. The feeling was that AND should be replaced by OR/AND. However, she showed the reference from the text book (Moss and Adams). I was unable to get the original article by Kuhne et al. If anyone has any recall or has the original article, I request you to pass it on for the benefit of others.
LOCATION, LOCATION, LOCATION
I am yet to find a person who has completely understood the VSDs! This simple looking lesion can pose so many surprises, that the moment you get comfortable with it, a new problem, hitherto not experienced, pops up. In one of our discussions, our senior surgeon opined that the outlet VSDs have development of early PVRI compared to VSDs in other locations. The opinion was seconded by others. I could not recall any statement of this sort in the texts. I presumed the probable reason to be the proximity with pulmonic valve. It is likely that the hemophysiology of outlet VSDs simulate AP Windows to a certain extent. Is my presumption correct? If anyone can come with proper explanation with proof, you will be rewarded by a special mention in the blog; your name would be written in CAPITAL LETTERS!!
BLUE OR NOT
We were coursing through some of the interesting cases seen off late. We do have a series of single pulmonary arteries unpublished yet. One of such cases was a hemitruncus with intact septae. The question was: would this patient ever by cyanosed? If yes, why and how? I was emphatic that such patients would never have a cardiac cyanosis. The pulmonary artery exposed to the high pressure would eventually develop high PVRI and restricts the blood flow. The entire right heart output is going to single lung and it may eventually develop high PVRI. Till then, the patient should be pink. After bilateral high PVRI, the patient may have signs of low cardiac output and failure rather than cyanosis, unless the PFO opens up or pulmonary AV fistulae develop. Hence, clinically, the loudness of S2 would determine the status of the patient. Soft S2 shows patient has at least one good lung and loud P2 shows the development of high PVRI in the hitherto good lung. However, there were some arguments against my logic. What is your opinion? We may not have seen such a scenario, but may logically conclude the outcome. Let me know your takes on this issue.
This scenario marks the end of another post. Let me get the suggestions and answers to all these queries. Put them in the comments box or mail them to drkiranvs@gmail.com Let’s see how best this platform can be utilised for the purpose of mutual learning.
Regards
Kiran
ಮಂಗಳವಾರ, ಮಾರ್ಚ್ 9, 2010
Welcome to NH blog. This is Dr Kiran.
From last post, we decided to put some anecdotes which carry a greater meaning than what meets the eye. In the attempt, I have picked up this small story one of my friends told me once. She also had no recall where she heard the story. It may very well be getting transmitted verbally from many. If this story is in print anywhere, I would be indebted to know the same from the readership.
The story goes something like this:
A board hung on a pet store caught the attention of this young boy of nine.
PUPPIES FOR SALE
Boy slowly went into the shop and smiled at the plump owner.
“How much are the puppies?”
“It would be Rs 300/- each. We have left with only seven more. Have cash or wanna come back with dad?”
The boy meddled with his pockets, brought out some change and counted.
“I have Rs 45/- only now. Can I see the puppies?”
The store owner was a kind man. “Come on in!” he said.
As the boy got in, he found seven cute creamish puppies running hither and thither. A smile laced his face. To his surprise, he found the eighth puppy limping along.
“I heard seven. But they are eight out there!”
The shop keeper looked down the place and said:
“Oh, that’s a defective one. We showed him to the doctor. This puppy has some problem in legs by birth. I have not included him in the list”
“Will he always limp like that?”
“Yes, my boy. Thats why I did not tell you about him”
“I want to buy him!” the boy said.
“No point, child. I don’t even have him in the list. I don’t want to sound cheating a little boy. If you still think you want him, I will give him to you for free.”
The boy’s face grew red. He sounded angry. “Please don’t use the word ‘free’. I don’t want him free. I want to buy him. He is worth the same as all his other siblings. You please keep this Rs 45 as advance and book him for me. I will send my father to pay the rest.”
The shop owner was perplexed. He also had the admiration for this small boy at the same time. He tried to convince the boy.
“I think you should consult your parents before making this decision. You see, this puppy is never going to be a normal, playful one. You will never be able to take him out for fun. There is no chance of you jumping and playing with him.
The boy smiled, “You are wrong”. He slowly pulled his pant up. It showed steel braces around his deformed leg. “It is not about this puppy. Even I can’t run around and jump either. We make a pair because we can understand each other!”
The shop-owner was speechless.
It takes lot of empathy to understand the feelings of patients’ companions. It becomes glaring when the tables turn. I remember our erstwhile boss writing to all of us when her father was in the ICU of a prestigious hospital at Hyderabad. She came back after the demise of her dad and was so full of new ideas about patient care and communication. She could feel for all the parents waiting in the lobby just to hear something about their patient by one of the personnel from inside. She emphasized the need for more frequent, easy and meaningful communications with patient party. She urged the importance of supporting the family at the times of distress and impending death. She conceived the idea of keeping the patient happy till the last moment by creating an atmosphere of solace for everyone. She suggested that we should allow the parents to stay bedside during the last moments of the child and the mutual satisfaction that can be provided when the parents can hold the baby’s hands in those moments. She mooted the proposal of making the end as beautiful as possible for the tender hearts.
Not that our brilliant boss could not have thought of all these issues prior. She probably had in bits and pieces. However, everything got crystallised into a solid structure when she went through the experience with tables turned around. She could empathize with her patients better after her experience with her father’s demise. One example of how a committed person would see the changes around with a positive outlook.
Let me know your views on it. Also, if you have read or heard of small anecdotes, please send them to me. Your contribution would be promptly acknowledged!
With this, let us get back to our regular feature: Challenging scenarios.
IS THIS TRUNCUS POSSIBLE?
I remember sometime back when a cardiologist from a small volume centre presented Truncus arteriosus with intact interventricular septum. We were smiling at the possible embryological nonsense. The shock was on us when we saw a baby with same diagnosis. Our surgical team questioned the well being of cardiology team when we mentioned this to them. We showed them the echo images to stun them. Our enthusiastic fellow, Dr Karunakar had already gone a step ahead and pulled out a couple of articles from his bag on the previous case reports of Truncus with intact IVS! The question was of the management. This 2-month-old was not very well at presentation. The surgeon went through the literature and had a couple of plans for him based on the anatomy on the table. Sadly, the baby did not make it to surgery and expired in the ICU. This is the first time we had come across any Truncus with intact IVS (we have operated 115 cases of truncus so far at NH). Please let me know if you have seen a truncus with intact IVS and how it was managed.
UNANSWERED McGOON
This has been a perpetual question that might have haunted generations. Probably, I have posted it earlier also. When one of the pulmonary arteries is far smaller than the other and the McGoon’s ratio becomes acceptable, what is the approach to be taken? We often see the surgical and cardiology teams split on the issue. What are the opinions from other centres? Please let me know your expertise in it.
WHOLE OR HOLE?
We had an eight year old with DORV, d-malposed great arteries, VSD, pulmonary atresia and intact IAS. This child saturated 78%. Although there were 2 good sized ventricles, the VSD was not routable. The question was whether to create a BTT shunt or to go for a BD Glenn shunt. The house was spilt on the decision. One half felt that the BTT shunt might be better, as BD Glenn has long term issues. The other half felt argued that the volume load on the ventricle by a BTT shunt would be detrimental on a long run and BD Glenn would serve more than one purpose. It was decided to measure the PA pressures on the table and go with BD Glenn if OK; otherwise to settle for BTT shunt. The other interesting question was – whether an ASD would be required in this case? Since the aorta was more committed to RV and VSD was just reasonable subpulmonic type, should this baby have an ASD to compliment mixing? This was a tough question to answer, as the surgeries proposed were off-pump and to create an ASD, one need to go on-pump. The surgical team could not recall any similar situation from their kitty of experience. Neither was the cardiology team sure of the answer. If anyone had such experience, please enlighten us.
BEHAVIOUR: BEFORE AND AFTER
We had a couple of children with VSD, PS, small RV and had gone through BD Glenn shunt few years back. Now, they returned with progressive restriction of VSD and symptoms. RV was suprasystemic on cath study. The RV EDP had gone up beyond the permissible level for Fontan completion. Although the Glenn shunt was still partially functioning, it was probably a matter of time. How should one go about such situations? Creation of a larger ASD may serve them for few more years and not a long term solution. Is there anything like enlarging or creating a VSD? The surgical team quoted “Prohibitive risk”. Is it the final word? Is there anything that can better such situations? Please tell us about your experiences.
ORTHOTOPY Vs HETEROTOPY
An interesting observation was quoted by one of our senior cardiologists, Dr PV Suresh. Amidst his ever-flowing OPD patients, he had observed the natural history of pulmonary homografts in Rastelli Vs Ross procedures. His observations showed that the Ross homografts behave far superior to the Rastelli homografts. He was seeking the possible explanations for this. The surgical team was represented by Dr Shekar Rao, who has exhaustive experience in both the procedures. Apart from the individual magnitude of both procedures and patient selection, Dr Shekar Rao quoted the advantages of orthotopic positioning of Ross homograft and possible disadvantages of heterotopic positioning of Rastelli homgrafts. The discussion was interesting for senior group and eye-opening for the freshers. We ended up understanding the hemodynamic variations of a 3-D angulation of cardiac structures. It is always emphasized that we cardiologist should have a mental 3-D reconstruction of anatomy with the 2-D data what we get from conventional imaging. This time, we could gauge the 3-D physiology also! We are indebted to both our senior consultants for the new light. Not to boast of ourselves, that’s probably what makes NH a sought after place for students!!
END OF AN ERA
After going through a series of emotional tides, the NH team is settling now. Dr Sunita Maheshwari, our beloved “Boss” decided to quit her post from NH to make space for her dreams. The decision was greeted with surprise, anguish, shock, anger, sorrow and mix of emotions from other team members. The Fellows were not very happy with the development, although it does not affect them on a long term. Such was the gravity created within. On the day of commemoration of a decade of Pediatric cardiology in NH, we also witnessed the handing over of charge to Dr PV Suresh. Although Dr Sunita has promised to come once a week for teaching, we know it is not long term happening. Personally, I feel that Dr Sunita’s achievement lies in the fact that she has prepared the department for her absence. It is no doubt the sign of a leader with a positive vision. Now, it is left for the team to honour her by achieving greater heights despite her absence. That’s one way I think we can thank her. We all wish for an uninterrupted flow of her creative energies and scaling of greater heights she is ever capable of. Adieu.
That brings us to the end of another post. I am yet to receive any comments for the new effort. Please let me know if it is worth continuing. Come out with any better formats for presentation. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership. I hope to get more frequent henceforth.
Regards
Kiran
From last post, we decided to put some anecdotes which carry a greater meaning than what meets the eye. In the attempt, I have picked up this small story one of my friends told me once. She also had no recall where she heard the story. It may very well be getting transmitted verbally from many. If this story is in print anywhere, I would be indebted to know the same from the readership.
The story goes something like this:
A board hung on a pet store caught the attention of this young boy of nine.
PUPPIES FOR SALE
Boy slowly went into the shop and smiled at the plump owner.
“How much are the puppies?”
“It would be Rs 300/- each. We have left with only seven more. Have cash or wanna come back with dad?”
The boy meddled with his pockets, brought out some change and counted.
“I have Rs 45/- only now. Can I see the puppies?”
The store owner was a kind man. “Come on in!” he said.
As the boy got in, he found seven cute creamish puppies running hither and thither. A smile laced his face. To his surprise, he found the eighth puppy limping along.
“I heard seven. But they are eight out there!”
The shop keeper looked down the place and said:
“Oh, that’s a defective one. We showed him to the doctor. This puppy has some problem in legs by birth. I have not included him in the list”
“Will he always limp like that?”
“Yes, my boy. Thats why I did not tell you about him”
“I want to buy him!” the boy said.
“No point, child. I don’t even have him in the list. I don’t want to sound cheating a little boy. If you still think you want him, I will give him to you for free.”
The boy’s face grew red. He sounded angry. “Please don’t use the word ‘free’. I don’t want him free. I want to buy him. He is worth the same as all his other siblings. You please keep this Rs 45 as advance and book him for me. I will send my father to pay the rest.”
The shop owner was perplexed. He also had the admiration for this small boy at the same time. He tried to convince the boy.
“I think you should consult your parents before making this decision. You see, this puppy is never going to be a normal, playful one. You will never be able to take him out for fun. There is no chance of you jumping and playing with him.
The boy smiled, “You are wrong”. He slowly pulled his pant up. It showed steel braces around his deformed leg. “It is not about this puppy. Even I can’t run around and jump either. We make a pair because we can understand each other!”
The shop-owner was speechless.
It takes lot of empathy to understand the feelings of patients’ companions. It becomes glaring when the tables turn. I remember our erstwhile boss writing to all of us when her father was in the ICU of a prestigious hospital at Hyderabad. She came back after the demise of her dad and was so full of new ideas about patient care and communication. She could feel for all the parents waiting in the lobby just to hear something about their patient by one of the personnel from inside. She emphasized the need for more frequent, easy and meaningful communications with patient party. She urged the importance of supporting the family at the times of distress and impending death. She conceived the idea of keeping the patient happy till the last moment by creating an atmosphere of solace for everyone. She suggested that we should allow the parents to stay bedside during the last moments of the child and the mutual satisfaction that can be provided when the parents can hold the baby’s hands in those moments. She mooted the proposal of making the end as beautiful as possible for the tender hearts.
Not that our brilliant boss could not have thought of all these issues prior. She probably had in bits and pieces. However, everything got crystallised into a solid structure when she went through the experience with tables turned around. She could empathize with her patients better after her experience with her father’s demise. One example of how a committed person would see the changes around with a positive outlook.
Let me know your views on it. Also, if you have read or heard of small anecdotes, please send them to me. Your contribution would be promptly acknowledged!
With this, let us get back to our regular feature: Challenging scenarios.
IS THIS TRUNCUS POSSIBLE?
I remember sometime back when a cardiologist from a small volume centre presented Truncus arteriosus with intact interventricular septum. We were smiling at the possible embryological nonsense. The shock was on us when we saw a baby with same diagnosis. Our surgical team questioned the well being of cardiology team when we mentioned this to them. We showed them the echo images to stun them. Our enthusiastic fellow, Dr Karunakar had already gone a step ahead and pulled out a couple of articles from his bag on the previous case reports of Truncus with intact IVS! The question was of the management. This 2-month-old was not very well at presentation. The surgeon went through the literature and had a couple of plans for him based on the anatomy on the table. Sadly, the baby did not make it to surgery and expired in the ICU. This is the first time we had come across any Truncus with intact IVS (we have operated 115 cases of truncus so far at NH). Please let me know if you have seen a truncus with intact IVS and how it was managed.
UNANSWERED McGOON
This has been a perpetual question that might have haunted generations. Probably, I have posted it earlier also. When one of the pulmonary arteries is far smaller than the other and the McGoon’s ratio becomes acceptable, what is the approach to be taken? We often see the surgical and cardiology teams split on the issue. What are the opinions from other centres? Please let me know your expertise in it.
WHOLE OR HOLE?
We had an eight year old with DORV, d-malposed great arteries, VSD, pulmonary atresia and intact IAS. This child saturated 78%. Although there were 2 good sized ventricles, the VSD was not routable. The question was whether to create a BTT shunt or to go for a BD Glenn shunt. The house was spilt on the decision. One half felt that the BTT shunt might be better, as BD Glenn has long term issues. The other half felt argued that the volume load on the ventricle by a BTT shunt would be detrimental on a long run and BD Glenn would serve more than one purpose. It was decided to measure the PA pressures on the table and go with BD Glenn if OK; otherwise to settle for BTT shunt. The other interesting question was – whether an ASD would be required in this case? Since the aorta was more committed to RV and VSD was just reasonable subpulmonic type, should this baby have an ASD to compliment mixing? This was a tough question to answer, as the surgeries proposed were off-pump and to create an ASD, one need to go on-pump. The surgical team could not recall any similar situation from their kitty of experience. Neither was the cardiology team sure of the answer. If anyone had such experience, please enlighten us.
BEHAVIOUR: BEFORE AND AFTER
We had a couple of children with VSD, PS, small RV and had gone through BD Glenn shunt few years back. Now, they returned with progressive restriction of VSD and symptoms. RV was suprasystemic on cath study. The RV EDP had gone up beyond the permissible level for Fontan completion. Although the Glenn shunt was still partially functioning, it was probably a matter of time. How should one go about such situations? Creation of a larger ASD may serve them for few more years and not a long term solution. Is there anything like enlarging or creating a VSD? The surgical team quoted “Prohibitive risk”. Is it the final word? Is there anything that can better such situations? Please tell us about your experiences.
ORTHOTOPY Vs HETEROTOPY
An interesting observation was quoted by one of our senior cardiologists, Dr PV Suresh. Amidst his ever-flowing OPD patients, he had observed the natural history of pulmonary homografts in Rastelli Vs Ross procedures. His observations showed that the Ross homografts behave far superior to the Rastelli homografts. He was seeking the possible explanations for this. The surgical team was represented by Dr Shekar Rao, who has exhaustive experience in both the procedures. Apart from the individual magnitude of both procedures and patient selection, Dr Shekar Rao quoted the advantages of orthotopic positioning of Ross homograft and possible disadvantages of heterotopic positioning of Rastelli homgrafts. The discussion was interesting for senior group and eye-opening for the freshers. We ended up understanding the hemodynamic variations of a 3-D angulation of cardiac structures. It is always emphasized that we cardiologist should have a mental 3-D reconstruction of anatomy with the 2-D data what we get from conventional imaging. This time, we could gauge the 3-D physiology also! We are indebted to both our senior consultants for the new light. Not to boast of ourselves, that’s probably what makes NH a sought after place for students!!
END OF AN ERA
After going through a series of emotional tides, the NH team is settling now. Dr Sunita Maheshwari, our beloved “Boss” decided to quit her post from NH to make space for her dreams. The decision was greeted with surprise, anguish, shock, anger, sorrow and mix of emotions from other team members. The Fellows were not very happy with the development, although it does not affect them on a long term. Such was the gravity created within. On the day of commemoration of a decade of Pediatric cardiology in NH, we also witnessed the handing over of charge to Dr PV Suresh. Although Dr Sunita has promised to come once a week for teaching, we know it is not long term happening. Personally, I feel that Dr Sunita’s achievement lies in the fact that she has prepared the department for her absence. It is no doubt the sign of a leader with a positive vision. Now, it is left for the team to honour her by achieving greater heights despite her absence. That’s one way I think we can thank her. We all wish for an uninterrupted flow of her creative energies and scaling of greater heights she is ever capable of. Adieu.
That brings us to the end of another post. I am yet to receive any comments for the new effort. Please let me know if it is worth continuing. Come out with any better formats for presentation. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership. I hope to get more frequent henceforth.
Regards
Kiran
ಭಾನುವಾರ, ಫೆಬ್ರವರಿ 21, 2010
This is Dr.Kiran welcoming the readers to NH blog. The blog aims to put something "old and fresh" of Pediatric Cardiology everytime and invites the readership to actively participate in the dialogue process.
Let me render my apologies to begin with. It has been more than one month that I have posted anything. I was a bit busy with an article for publication, under a strict ultimatum! Howsoever small the readership might be, I owe this explanation to them. Hence this.
So far, we have seen something about the historical developments, Nobel prize winners and historical developments of drugs: all in relation to Pediatric Cardiology.
I had run out of the ideas and asked the readers on some suggestions on the topics of possible interest. As usual, I have got none! So, I have tried to do something which was suggested sometime back by one of the very good friends and a fabulous team-member, Dr Vishal Changela. He strongly felt that some personal medical anecdotes about our professional relationship with patients should be put up for others to read. In the absence of any other better things to write, I am trying this from now on.
Long back, I had heard a short story written by one of the greatest short-story writers of Kannada, the legendary Dr Masti Venkatesha Iyengar. It is a simple village story. One evening in one of the villages neighbouring a forest, a cowherd realises that a buffalo and its calf are missing. Since it is already dark, they fear entering the forest in search of the missing duo. He keeps his fingers crossed and waits till the dawn. A search party with the armamentarium enters the forest carefully. After a couple of hours of search in the deep forest, they hear the hunger cries of a calf. They start towards the sound and find the same calf of the buffalo safe and secure. In a short distance from this, they find the wounded buffalo in a pool of blood, breathing heavily. To their shock, they find the paw marks of tiger moving away from the spot with blood splattered all along its course, disappearing into the heart of the forest. They immediately realised the entire sequence of events. The calf must have wandered into the forest unknowingly in the late evening and its mother must have followed it to safeguard the kid. A tiger must have attacked the calf and the docile buffalo had fought the mighty tiger tooth and nail throughout the night to save its baby. It finally succeeded at the cost of its own life, driving the deadly beast wounded and away. Just imagining a tame buffalo fighting a ferocious tiger in the night to protect its calf keeping its own life on stake was nerve wrecking and emotions filled the souls of those poor peasants. The villagers carried the wounded buffalo and the calf back to the village. The buffalo eventually died and the villagers buried it with full honours that befitted a brave person and had a small monument built in the memory of its love and courage. This true story stands as a phenomenal symbol of selfless sacrifice of a mother for its child.
The story brings about various kinds of emotions in the reader. Few imagine the scenario and feel for the motherhood. Few imagine themselves in the scenario and try emoting. Few extrapolate the real life situations and think of cross references. What comes out an anecdote is your experience and empathy inside. We all feel for the sacrifice of the buffalo largely because of the courage this helpless, docile and mute animal showed at the time of danger and crisis to its calf. It is probably the basic nature of this animal that stands contrast to the courage it displayed, which fills the mind.
We do see situations in the hospital similar to the one the anecdotal buffalo underwent. We see parents who would sacrifice what all they can to get their children treated. We see them selling the last piece of jewellery, spreading their hand in front of the last available donor, saving money by choosing the least expensive accommodation for themselves or taking just one lunch and so on when the child is admitted in the hospital. All the gruesome effort happens despite accepting the risk and keeping the outcome sceptical. The sacrifice appears to be hanging only to a 4-letter word: HOPE. I am often reminded of the anecdotal buffalo whenever I come across such situations. The scenario of the otherwise helpless buffalo is no different from helpless parents here. The buffalo fought with mighty, cruel tiger and the parents here fight with mightier, more cruel and more than that - an unseen destiny. I feel the sacrifices on both occasions are no less than each other. The first scenario got noticed. But the latter goes unnoticed all the time.
These are the kind of anecdotes that we would like to present. It need not always be tragical. Some triumphs can also be interspersed. At finale, what re-shines is the humanity. The Mahabharata says, “there needs to be only one religion and one principle in the world: compassion to the fellow life”. It is said that the humanity re-lives every time it triumphs. If you have any such anecdotes, please send them to me via email to drkiranvs@gmail.com I shall acknowledge your contribution and give full credit to you for your story!!
I shall get back to our regular feature: Interesting and perplexing case scenarios
TO OR NOT TO TIGHTEN
When the pre-Glenn shunt cath procedure is done, we often see PA pressures being borderline high, but all other parameters would favour Glenn shunt. In such scenarios, few suggest tightening of MPA and going ahead with Glenn. It may get the circuit OK for Glenn, but may reduce the SO2. Is this acceptable? Isn’t good SO2 one of the objectives of Glenn? By tightening the MPA, do we always achieve the Glenn suitability? If anyone found answers for these, please let me know.
BALLOONING IN REGURGITATION
In cases of severe Aortic stenosis with moderate aortic regurgitation, can we consider ballooning? We had a 13-year-old with this scenario. His aortic annulus was very small and looked mildly dysplastic. Surgical team felt that placing a good sized aortic prosthesis was not possible. Doing a Konno was considered very risky. Annuli were not suitable for Ross. In such cases, can we contemplate controlled balloon dilatation, accepting the eventual AR? Our senior consultant was of the opinion that free AR would be very dangerous in such children and balloon should not be attempted. Is the natural history in this subset any different from any kind of intervention? What would you do in such cases? Please put your comments on it.
Qp OR Qep?..
Numbers are funny. We have seen hair-splitting episodes on the basis of numbers generated after gruelling procedures! Whether operability or fitness for univentricular procedures, numbers do play a vital role in this field. During the combined cath meeting, the house is split many a times on this factor alone. We often find children cathed for operability. When the shunt is bidirectional, as per the standard references, we use the effective shunt – Qep. The Qep, many times, is significantly different from the Qp. In all such cases, should we use Qep or Qp as the denominator for calculating the PVRI? This alone can make the difference for the patient. We had a 7-year-old with VSD, PDA and PAH, whose operability was doubtful on the clinical grounds. Basic investigations could not be deciding. Cath showed reasonable Qp/Qs, but the PVRI was marginally high. Our team felt that the child should be operated, as this might be his last chance. Surgical team quoted the higher number PVRI and mooted inoperability. We were no sure how much to believe the numbers when the denominator was not fully certain. Is there a correction factor to the formulae when Qep is involved? Let me know your experience and literature back up if any.
PLACING THE BAND
An interesting analogy was discussed by the surgical team in one of the meetings. It is probably not textbook written and might be more experience oriented. While witnessing the angio images of a child who had undergone a PA banding as an infant in a hospital abroad, one of the senior surgeons commented that the said PA band was meant for a future single pump physiology. We were surprised how he could be so certain, as he was seeing the details of the patient for the first time! Then the surgeon pointed out that the PA band was placed close to the pulmonary valve, which is done to prevent distortion to the branch PAs. Similarly, he said, the PA bands are placed close to the bifurcation if a future 2-pump repair is contemplated. We were not certain if that was the rule, as we were considering both options in the present patient. Other members of the surgical team agreed with this. However, I could not recall reading about this anywhere, nor my cardiology colleagues. If anyone has read something about this, please enlighten us.
SINGLE VENTRICLE: WOULD LV/RV BE A FACTOR?
This was an interesting one. We had a 5-year-old with tricuspid atresia IIIC, who had undergone a PA band as an infant and saturating 88% at present. We had suggested single pump repair for the child. She fulfilled the criteria for single pump repair. However, the surgical team felt that the child can be followed up, as the saturations are OK. We argued that the long-term ventricular load should be the main consideration for present surgery. At this point, one of the senior surgeons commented that he would have taken it if it were to be a mitral atresia instead of tricuspid! He was of the opinion that the volume load on the ventricle would be a factor only when the pumping single chamber is RV. As this patient would have LV as the pumping single chamber, volume load should not be a consideration for present intervention. In any of the criteria of single ventricle, this factor does not find a place. We were using this logic for long term prognosis of single ventricle repair, but what our surgeon told appeared new. It may sound logical, but is there any consensus among the surgeons on this? How would you tackle this situation and argument? Please let me know your take on this.
TWO IN ONE?!
We had an infant with dTGA, ASD, small VSD and severe peripheral PS. The branch PAs appeared to have a tortuous course. The echo study alone was not enough to understand the branch PA anatomy. It was not very clear if they had multiple constrictions. From our side, we were not in consensus on the plan. We decided to place the scenario in the meeting. We showed the echo images and asked the surgeons to comment on it. Although it was decided for a close follow up at present, one of the surgeons felt that the LeCompte manoeuvre done during arterial switch can straighten the branch PAs and they may not require any additional intervention! This was hitherto unheard of! Again, sounded logical, but is it the fact? One can understand that the logic would work if the branch PAs have just folded upon themselves. Would the same logic apply if there are anatomical constrictions in the branch PAs? If anyone had any similar experiences earlier, please let us know the outcome and the way you went ahead with the plan.
That brings us to the end of the present post. I have tried something different; totally different from my areas of strength! If there are any differences of opinion, please let me know. If you think any better formats for presentation, please suggest. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership
Regards
Kiran
Let me render my apologies to begin with. It has been more than one month that I have posted anything. I was a bit busy with an article for publication, under a strict ultimatum! Howsoever small the readership might be, I owe this explanation to them. Hence this.
So far, we have seen something about the historical developments, Nobel prize winners and historical developments of drugs: all in relation to Pediatric Cardiology.
I had run out of the ideas and asked the readers on some suggestions on the topics of possible interest. As usual, I have got none! So, I have tried to do something which was suggested sometime back by one of the very good friends and a fabulous team-member, Dr Vishal Changela. He strongly felt that some personal medical anecdotes about our professional relationship with patients should be put up for others to read. In the absence of any other better things to write, I am trying this from now on.
Long back, I had heard a short story written by one of the greatest short-story writers of Kannada, the legendary Dr Masti Venkatesha Iyengar. It is a simple village story. One evening in one of the villages neighbouring a forest, a cowherd realises that a buffalo and its calf are missing. Since it is already dark, they fear entering the forest in search of the missing duo. He keeps his fingers crossed and waits till the dawn. A search party with the armamentarium enters the forest carefully. After a couple of hours of search in the deep forest, they hear the hunger cries of a calf. They start towards the sound and find the same calf of the buffalo safe and secure. In a short distance from this, they find the wounded buffalo in a pool of blood, breathing heavily. To their shock, they find the paw marks of tiger moving away from the spot with blood splattered all along its course, disappearing into the heart of the forest. They immediately realised the entire sequence of events. The calf must have wandered into the forest unknowingly in the late evening and its mother must have followed it to safeguard the kid. A tiger must have attacked the calf and the docile buffalo had fought the mighty tiger tooth and nail throughout the night to save its baby. It finally succeeded at the cost of its own life, driving the deadly beast wounded and away. Just imagining a tame buffalo fighting a ferocious tiger in the night to protect its calf keeping its own life on stake was nerve wrecking and emotions filled the souls of those poor peasants. The villagers carried the wounded buffalo and the calf back to the village. The buffalo eventually died and the villagers buried it with full honours that befitted a brave person and had a small monument built in the memory of its love and courage. This true story stands as a phenomenal symbol of selfless sacrifice of a mother for its child.
The story brings about various kinds of emotions in the reader. Few imagine the scenario and feel for the motherhood. Few imagine themselves in the scenario and try emoting. Few extrapolate the real life situations and think of cross references. What comes out an anecdote is your experience and empathy inside. We all feel for the sacrifice of the buffalo largely because of the courage this helpless, docile and mute animal showed at the time of danger and crisis to its calf. It is probably the basic nature of this animal that stands contrast to the courage it displayed, which fills the mind.
We do see situations in the hospital similar to the one the anecdotal buffalo underwent. We see parents who would sacrifice what all they can to get their children treated. We see them selling the last piece of jewellery, spreading their hand in front of the last available donor, saving money by choosing the least expensive accommodation for themselves or taking just one lunch and so on when the child is admitted in the hospital. All the gruesome effort happens despite accepting the risk and keeping the outcome sceptical. The sacrifice appears to be hanging only to a 4-letter word: HOPE. I am often reminded of the anecdotal buffalo whenever I come across such situations. The scenario of the otherwise helpless buffalo is no different from helpless parents here. The buffalo fought with mighty, cruel tiger and the parents here fight with mightier, more cruel and more than that - an unseen destiny. I feel the sacrifices on both occasions are no less than each other. The first scenario got noticed. But the latter goes unnoticed all the time.
These are the kind of anecdotes that we would like to present. It need not always be tragical. Some triumphs can also be interspersed. At finale, what re-shines is the humanity. The Mahabharata says, “there needs to be only one religion and one principle in the world: compassion to the fellow life”. It is said that the humanity re-lives every time it triumphs. If you have any such anecdotes, please send them to me via email to drkiranvs@gmail.com I shall acknowledge your contribution and give full credit to you for your story!!
I shall get back to our regular feature: Interesting and perplexing case scenarios
TO OR NOT TO TIGHTEN
When the pre-Glenn shunt cath procedure is done, we often see PA pressures being borderline high, but all other parameters would favour Glenn shunt. In such scenarios, few suggest tightening of MPA and going ahead with Glenn. It may get the circuit OK for Glenn, but may reduce the SO2. Is this acceptable? Isn’t good SO2 one of the objectives of Glenn? By tightening the MPA, do we always achieve the Glenn suitability? If anyone found answers for these, please let me know.
BALLOONING IN REGURGITATION
In cases of severe Aortic stenosis with moderate aortic regurgitation, can we consider ballooning? We had a 13-year-old with this scenario. His aortic annulus was very small and looked mildly dysplastic. Surgical team felt that placing a good sized aortic prosthesis was not possible. Doing a Konno was considered very risky. Annuli were not suitable for Ross. In such cases, can we contemplate controlled balloon dilatation, accepting the eventual AR? Our senior consultant was of the opinion that free AR would be very dangerous in such children and balloon should not be attempted. Is the natural history in this subset any different from any kind of intervention? What would you do in such cases? Please put your comments on it.
Qp OR Qep?..
Numbers are funny. We have seen hair-splitting episodes on the basis of numbers generated after gruelling procedures! Whether operability or fitness for univentricular procedures, numbers do play a vital role in this field. During the combined cath meeting, the house is split many a times on this factor alone. We often find children cathed for operability. When the shunt is bidirectional, as per the standard references, we use the effective shunt – Qep. The Qep, many times, is significantly different from the Qp. In all such cases, should we use Qep or Qp as the denominator for calculating the PVRI? This alone can make the difference for the patient. We had a 7-year-old with VSD, PDA and PAH, whose operability was doubtful on the clinical grounds. Basic investigations could not be deciding. Cath showed reasonable Qp/Qs, but the PVRI was marginally high. Our team felt that the child should be operated, as this might be his last chance. Surgical team quoted the higher number PVRI and mooted inoperability. We were no sure how much to believe the numbers when the denominator was not fully certain. Is there a correction factor to the formulae when Qep is involved? Let me know your experience and literature back up if any.
PLACING THE BAND
An interesting analogy was discussed by the surgical team in one of the meetings. It is probably not textbook written and might be more experience oriented. While witnessing the angio images of a child who had undergone a PA banding as an infant in a hospital abroad, one of the senior surgeons commented that the said PA band was meant for a future single pump physiology. We were surprised how he could be so certain, as he was seeing the details of the patient for the first time! Then the surgeon pointed out that the PA band was placed close to the pulmonary valve, which is done to prevent distortion to the branch PAs. Similarly, he said, the PA bands are placed close to the bifurcation if a future 2-pump repair is contemplated. We were not certain if that was the rule, as we were considering both options in the present patient. Other members of the surgical team agreed with this. However, I could not recall reading about this anywhere, nor my cardiology colleagues. If anyone has read something about this, please enlighten us.
SINGLE VENTRICLE: WOULD LV/RV BE A FACTOR?
This was an interesting one. We had a 5-year-old with tricuspid atresia IIIC, who had undergone a PA band as an infant and saturating 88% at present. We had suggested single pump repair for the child. She fulfilled the criteria for single pump repair. However, the surgical team felt that the child can be followed up, as the saturations are OK. We argued that the long-term ventricular load should be the main consideration for present surgery. At this point, one of the senior surgeons commented that he would have taken it if it were to be a mitral atresia instead of tricuspid! He was of the opinion that the volume load on the ventricle would be a factor only when the pumping single chamber is RV. As this patient would have LV as the pumping single chamber, volume load should not be a consideration for present intervention. In any of the criteria of single ventricle, this factor does not find a place. We were using this logic for long term prognosis of single ventricle repair, but what our surgeon told appeared new. It may sound logical, but is there any consensus among the surgeons on this? How would you tackle this situation and argument? Please let me know your take on this.
TWO IN ONE?!
We had an infant with dTGA, ASD, small VSD and severe peripheral PS. The branch PAs appeared to have a tortuous course. The echo study alone was not enough to understand the branch PA anatomy. It was not very clear if they had multiple constrictions. From our side, we were not in consensus on the plan. We decided to place the scenario in the meeting. We showed the echo images and asked the surgeons to comment on it. Although it was decided for a close follow up at present, one of the surgeons felt that the LeCompte manoeuvre done during arterial switch can straighten the branch PAs and they may not require any additional intervention! This was hitherto unheard of! Again, sounded logical, but is it the fact? One can understand that the logic would work if the branch PAs have just folded upon themselves. Would the same logic apply if there are anatomical constrictions in the branch PAs? If anyone had any similar experiences earlier, please let us know the outcome and the way you went ahead with the plan.
That brings us to the end of the present post. I have tried something different; totally different from my areas of strength! If there are any differences of opinion, please let me know. If you think any better formats for presentation, please suggest. Put them in the comments box or mail them to drkiranvs@gmail.com I shall consider them seriously and get back to the readership
Regards
Kiran
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