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

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