Thanks to progress in cardiac surgery and cardiology, pediatric patients with complex congenital heart conditions who would previously have died are now reaching adulthood. Patients with transposition of the great arteries who have undergone atrial repair can present during follow-up with progression towards heart failure as a result of progressive systemic right ventricular failure. In this situation, heart transplantation is a possible therapeutic option. Anatomic abnormalities and the presence of intraatrial conduits ensure that transplantation must involve a number of technical modifications. Here, we present our experience during 1992-2004 with heart transplantations in four patients with transposition of the great arteries and atrial repair. There was no operative mortality. During follow-up (mean period, 75 months), there was one death due to chronic rejection. The other patients remain in New York Heart Association class I, with normally functioning grafts.