Pediatric Heart Expert Forum
Consequences of a Faulty Pulmonary Valve
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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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Consequences of a Faulty Pulmonary Valve

My son had complete repair of his classic TOF condition four months ago and his surgeon was able to save his valve. We just had an echo done yesterday and his valve is not functioning at 100%, but rather at 60%. His cardiologist said he will have to have a new valve put in when he's full grown, but by then, it will be a perfected science and not a dangerous procedure.  My question is what will he go through until his valve replacement?  Will he be tired easily?  Should we be careful with his activities?  Finally, what would his expected life expectancy most likely be?  He does not have any other conditions as far as we know and is otherwise quite a big and healthy baby. Thanks!
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Dear Araksi,

I cannot say exactly what your son will experience, as I have not evaluated him nor have I seen his studies.  If he has a significant leak in his pulmonary valve, but his right ventricle is functioning normally and he has no symptoms, he should do fine into adolescence or early adulthood.  What we see over time, though, is that with chronic moderate or severe pulmonary valve regurgitation, the right ventricle can dilate.  With that, you can see exercise intolerance and arrhythmias.  The onset of symptoms can vary, with some people experiencing symptoms early and others having no symptoms despite grossly abnormal heart function.  For now, we have not been limiting activities if his heart otherwise appears normal.  As well, since it sounds as if your son is an infant, I can say that infants and children are quite good at self-limiting their activities anyway (no matter what their heart looks like!).  If he is able to continue with appropriate pediatric cardiac, and eventually adult congenital heart disease, care, his lifespan should be able to reasonable into mid-adulthood, or even later.
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Jeffrey R Boris, M.D.Blank
The Children’s Hospital of Philadelphia
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