The purpose of this study was to review the outcome of pediatric heart recipients with uncorrectable congenital heart disease, failed corrective procedures, or intractable acquired cardiomyopathy. Between 1988 and 2005, cardiac transplantations were performed in 128 pediatric patients (59 girls and 69 boys) at the Heart-Center North-Rhine-Westphalia. Their ages varied between 1 week and 18 years (mean, 7.6 +/- 6.2 years). Underlying diseases were dilative cardiomyopathy in 93 cases and uncorrectable congenital heart disease in 35 cases. We diagnosed and observed 130 episodes of acute rejection in 85 patients (65.3% of patients had an acute rejection during the observation period); 71 patients were treated efficiently with steroid-pulse therapy. Monoclonal antibody OKT3 was administrated in 14 patients because of steroid-resistant acute donor organ rejection. Five of those 14 patients survived. The cumulative survival was 88% after 1 year and 68% after 10 years for all patients. Orthotopic heart transplantation is the ultimate treatment option for children with end-stage heart disease and shows an acceptable perioperative mortality rate as well as good long-term results.