Cardiac transplantation has become a more frequently used therapeutic modality for select cardiac pathology in infants and children. Since June 1986, 30 pediatric patients (19 male and 11 female) ranging in age from 4 days to 15 years (11 < or = 1 month old) have undergone orthotopic cardiac transplantation at our institution. Indications included idiopathic cardiomyopathy (n = 8), hypoplastic left heart syndrome (n = 13), and other forms of complex congenital heart disease (n = 9). There have been four operative and three late deaths only in the groups with hypoplastic left heart syndrome and other forms of complex congenital heart disease. Cumulative survival is 77% after a mean follow-up of 30 months (range, 6 to 77 months). Three operative deaths were attributable to pulmonary hypertension, and the other was due to pulmonary hemorrhage. Two late deaths were secondary to allograft rejection, and the third was due to infection. There has been uniform survival in the group with idiopathic cardiomyopathy, and intermediate-term survival rates are 78% and 62% in the groups with complex congenital heart diseases and hypoplastic left heart syndrome, respectively (p = 0.15). Although longer-term results are necessary, orthotopic cardiac transplantation appears to be an acceptable mode of therapy for endstage heart disease in the pediatric age group and technically can be performed despite complex malformations of the great arteries or atria.