Pediatric Heart Expert Forum
Fontan Procedure
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Questions in this forum are answered by pediatric cardiologists, cardiothoracic surgeons and anesthesiologists from The Children's Hospital of Philadelphia. This forum is for questions and support about pediatric heart problems, symptoms and topics such as heart murmurs, palpitations, fainting, chest pain, congenital heart defects (including management and intervention), fetal cardiology, adult congenital cardiology, arrhythmias and pre-participation athletic screening.

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Fontan Procedure

Hi

My neice has a congenital heart defects. She is two months old right now. Her condition has not been diagnosed fully as the doctors are waiting for a MRI to get a 3-d imagine. However we do know that she has TGA with two large VSD and plumonary artesia. She was symptom free for the first two  months but on her last checkup her saturation levels dropped and she started having a few spells. The medication given to her did not help and she just had her BT shunt procedure from which she is recovering well.

Her doctor has said that there are three options for correction: double switch, don't know the name of the second procedure but basically they don't switch the ventricles but close the VSD and do correct other problems or fontan. They have indicated that due to the size of her VSD she may need a fontan but they cant confirm without MRI.

What I want to know that if the doctors decide to go the fontan route should we get a second opinion?

Secondly what will her life be with fontan. I have heard mixed reviewed where some people say that life can be good and fontan can last for years where as some ppl say that you need a heart transplant. Do all patients with fontan procedure need a heart transplant? What is the % that do? Can she expected to have a good quality of life is she has a fontan?

Thanks

Preetika Joshi
773637_tn?1327450515
Dear Preetika,

Without evaluating her, I cannot say at all which would be the best option for her cardiac status.  I can say that, although the Fontan has been lifesaving for babies and children, and allowed them to reach adulthood, it is not a perfect solution.  There are many long term problems with it, including the ability to maintain the appropriate physiology necessary from a cardiopulmonary standpoint as well as intestinal and liver problems and endocrine problems.  Many of these children do end up with cardiac transplants.

If the decision is to get a Fontan, I would definitely get a second opinion.  However, with pulmonary atresia, it may be difficult to get a two ventricular repair out of her cardiac anatomy, especially if there is concern that one of the ventricles is inadequate.
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