Three hundred and forty-five pediatric patients underwent orthotopic heart transplantation at Loma Linda University Medical Center throughout August 1999. Seventy-five percent of these patients had the diagnosis of congenital heart disease as the primary indication for transplantation. Two hundred and fifty-seven were infants and of these, 91 were neonates. Forty-nine percent had the primary diagnosis of hypoplastic left heart syndrome or its equivalent. Thirty patients had variant situs and 15 of these had situs inversus. Seventy-seven children had undergone one or more previous cardiac and/or thoracic procedures. There appeared to be a survival advantage with neonatal as compared to infant heart transplants (77% vs. 63%). Technical considerations for reconstruction have made congenital heart transplantation a low perioperative risk factor as compared to the child with cardiomyopathy. Attention to (1) recipient selection; (2) adequate en bloc removal of the heart and accompanying vessels; (3) use of low-flow bypass techniques with limited circulatory rest; and (4) multidisciplinary management have been the fundamental approach at this institution. Although challenging, high perioperative survival and long-term success can be achieved with heart transplantation in patients with high-risk or inoperable congenital heart disease.