Orthotopic heart transplantation in a child with heterotaxia syndrome: a case report.

We report a case of a successful orthotopic heart transplantation in a seven year old boy with heterotaxia syndrome, d-transposition of the great arteries, rudimentary right ventricle, double inlet left ventricle, valvular pulmonary stenosis, left-sided peripheral pulmonary artery stenosis, deficient atrial septation, and left-sided superior and inferior vena cava. In this case of congenital heart defects, three specific surgical procedures were necessary to make heart transplantation feasible. A synthetic patch was interposed in the single atrium to create two separate atria. To handle the mismatch of the donor's and recipient's aortic diameters, the donor's aortic diameter was enlarged by including the aortic arch into the anastomosis. The left-sided superior vena cava was led to the right appendage by interposing a piece of autogenous aorta.