Wednesday, January 15, 2014

Dr Kiran welcomes everyone again to the blog of Pediatric cardiology department of Narayana Hrudayalaya.

I am not really sure if I can still use the term Hrudayalaya (meaning, home of heart), as the institute has deserted its use! Our hospital is now named Narayana Institute of Cardiac Sciences. But, I will continue with Hrudayalaya until I get comfortable with the new name! After all, what is there in a name; every hospital by any other name would smell the same! (with due apologies to William Shakespeare)

I came across an article in 2011 issue of Pediatric Cardiology journal on the utility of 3-D MRI in the coronary anatomy assessment for congenital heart diseases by Rajiah et al from Cleveland clinic, USA. The authors have done a commendable job of using whole heart MRI for coronary artery imaging in 112 CHD patients with mean age of 17 years. They have used a navigator-gated, whole-heart, 3D technique of MRI using 1.5 T machine to assess LAD, LCX and RCA. They have found that the coronary artery origins were visible in 99% with 7% of the patients having anomalies which included 35 patients of TOF, 5 of dTGA, 4 of ccTGA, 6 of suspected coronary anomaly and so on. The mean image acquisition time was 9 minutes amounting to 565 heartbeats. The authors claim that the study happens to be the largest and most comprehensive one using MRI to image the proximal coronary arteries in CHD. The learnings from the authors appears to have a significant impact in future of coronary imaging in CHD. This article is worth a read for anyone involved with management of CHD.

That brings us to the interesting leaning scenarios of this post.


Very often we come across children with significant cardiac defects mandating surgical intervention, but with just one functioning lung, be it hypoplasia or damage due to causes such as infection. The ipsilateral pulmonary artery would behave likewise. This non-functional lung not only imparts great stress on the other lung, but it also contributes to high resistance in the pulmonary arterial circuit. This jeopardizes the operability decisions. What is the optimal management in such cases? How to categorically assess the operability? Is surgical removal of non-functional portions of lung carry better outcome and prognosis? Should the cardiac surgical decisions on these patients be very aggressive? Please share your opinions and protocols followed at other centres.


Multiple terminologies are really confusing! Few claim that the Scimitar syndrome is actually called pulmonary venolobar syndrome. And, there are people on the opposite side to claim that both are different. We had an adolescent boy with CT reported as pseudohorseshoe lung! His angiogram showed us that the right lower lobe of the lung was getting its vascular supply through a collateral from abdominal aorta and its venous return going into right atrium. Chest radiogram showed heart displaced to right and lifted well above the right dome of diaphragm. After doing some vigorous literature search, we found the following learnings:
•    Horseshoe lung: congenital pulmonary malformation usually associated with scimitar. There is fusion of both pulmonary lobes from the posterobasal segments. The fusion is in the retrocardiac area, in front of the esophagus and thoracic aorta.
•    Pleural separation of pulmonary lobes distinguishes pseudohorseshoe appearance from a true horseshoe lung.
•    Scimitar syndrome is also known as hypogenetic lung syndrome. It is a part of the congenital pulmonary venolobar syndrome.

Those who seek more information on this can try Konen et al in 2003 issue of RadioGraphics on Congenital Pulmonary Venolobar Syndrome or Tosun et al in 2012 issue of Iranian Journal of Radiology on Congenital Pseudohorseshoe Lung Associated with Scimitar Syndrome.


It is not uncommon to see a child with Complete AV Canal defect with severe RVOT obstruction, resembling a tetralogy situation. This unique combination is often called Tet-Canal or Canal-Tet. Not only surgeon faces a greater challenge in this combination, there is also the question of the right timing. One school firmly believes in postponing the surgery to as later a date as possible, but the other maintains that the Tet-Canal surgery should not be delayed. What do the readers feel? What would be the ideal time to go in for definitive surgery in this subset of children, when the anatomical findings are in favour of complete correction? Please share your views.


One of the most difficult decisions for a surgeon is absence of plan B! Taking Ebsteins anomaly in a child with ccTGA, the surgeon often find himself in the cross-roads! The success of ccTGA repair would depend upon how successfully the Ebsteins anomaly is fixed and how much RV size is re-created. Since the latter is highly variable and the success cannot be instantaneously measured on the table, the decision to go ahead with anatomical correction of ccTGA would be practically risky, more so when the RV size is reasonably compromised. Adding to the woes is the lack of good plan B. What if the Ebsteins correction is suboptimal? This has always been intriguing to us. Please share your opinions and experiences.


Use of permanent pacemaker in the post-operative heart block is well established. However, it is the timing which has conflicting opinions. Few wait for 2 weeks, few for 10 days and so on. What is the ideal time? How long would be comfortable if the patient is hemodynamically stable? Please let us know your views.

That brings us to the end of this post. Please write in your comments. If you find any problem in posting comments, please feel free to mail it to my email id I shall post them on your behalf.




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