Should cardiac transplantation for congenital heart disease be delayed until adult age?

The number of pediatric heart transplantations for complex congenital heart disease has increased over the last years, but little experience has been reported in adolescent and adult populations. Between 1987 and 1992, 14 patients (mean age 33.2 years, range 14 to 51 years) were transplanted in our institution because of structural congenital heart disease (n = 9) or other rare disorders of the endomyocardial morphogenesis (n = 5). The main diagnosis included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypoplasia, double outlet right ventricle with transposition, left ventricular sinusoidal malformation and right ventricular dysplasia. In several cases there were additional intracardiac malformations, including ventricular septum defect, atrial septum defect as well as different forms of pulmonary stenosis. Seven patients had undergone one or more palliative repairs that consisted of modified Blalock-Taussig shunts, Glenn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atrioseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor cardiectomy was modified in order to include complete inflow and outflow tissue in the explant and transplantation could be performed without prosthetic material in all patients; deep hypothermic cardiac arrest was never necessary in this series. There was no early or late mortality after a mean follow-up of 37 months (range 4 to 74 months); postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results in all patients. Adolescent and adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)