Pediatric Heart Expert Forum
Degeorge Syndrome, pulmonary atresia, vsd, mapcas
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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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Degeorge Syndrome, pulmonary atresia, vsd, mapcas

My 4 yo granddaughter has already had three surgeries on her heart and the last one the doctors said if her mapcas didnt start growing she will die. She is now showing signs such as  blueness and fatigie and sleeping too much. Is there any specialist that works specifically with the mapcas? Our heart surgeon is great but that answer isnt acceptable. Is there any way to make them grow? Anything?
773637_tn?1327450515
Dear Shelley,

Pulmonary atresia with VSD (also known as tetralogy of Fallot with VSD) is a very difficult diagnosis to deal with.  In essence, the branch pulmonary arteries are often exceedingly small or even nonexistent, and the lungs are fed by multiple aorto-pulmonary collateral arteries (MAPCAs).  Typically what is attempted is to "unifocalize" these MAPCAs to get them all into the middle and to make them act like new pulmonary arteries.  The surgery does this initially, and then they often have cardiac catheterization with balloon angioplasty to try to dilate them with or without stenting.  Unfortunately, especially with DiGeorge syndrome, we have not been able to get either the MAPCAs or the native pulmonary arteries to grow.  Dr. Frank Hanley in San Francisco is known for his work with these patients.  However, there are some patients with whom even he has not been able to be successful.  The other potential option would be lung or heart/lung transplantation, which is a very extreme step in this case, and can be limited by the donor supply.
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Thank you so much for your research on this. Unfortunately, not a great diagnosis but this is what we have been told before. Thanks for the name of the doctor.
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