Congenital heart disease as an indication for heart transplantation accounts for a small number of the total heart transplant experience--less than 3% in most centers. We have performed heart transplantation in eight such patients, accounting for 4% of our total experience. All these patients had specific anatomic anomalies relevant to transplantation. None had morbidity directly related to their anatomic defect and subsequent transplant. Six of the eight had undergone no prior palliative or corrective repairs. One child had a left Blalock-Taussig shunt, and one had bilateral Glenn shunts and a Fontan repair. Technical considerations for orthotopic heart transplantation are described for transposition of the great arteries, left superior vena cava with and without bridging innominate vein, common atrium, presence of Blalock-Taussig shunt, bilateral Glenn shunts, and Fontan repair. Anatomic congenital heart disease is becoming a more frequent indication in heart transplantation. Heart transplantation in the presence of structural congenital heart disease may be technically challenging. Nevertheless, transplantation offers an effective therapeutic alternative for patients with end-stage congestive heart failure and congenital heart disease.