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.
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.