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 a nd 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 morphog enesis (n = 5). The main diagnoses included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypo plasia, double outlet right ventricle with transposition, left ventric ular sinusoidal malformation and right ventricular dysplasia. In sever al cases there were additional intracardiac malformations, including v entricular septum defect, atrial septum defect as well as different fo rms of pulmonary stenosis. Seven patients had undergone one or more pa lliative repairs that consisted of modified Blalock-Taussig shunts, Gl enn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atri oseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor c ardiectomy was modified in order to include complete inflow and outflo w 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 mortali ty after a mean follow-up of 37 months (range 4 to 74 months); postope rative echocardiography and cardiac catheterization demonstrated perfe ct anatomical and functional results in all patients. Adolescent and a dult patients with complex congenital cardiac diseases can be transpla nted with a very low perioperative risk, even after several prior oper ative procedures. The operative technique must be adapted to the malfo rmation; end-to-end bicaval anastomosis is advantageous, and extensive reconstruction of the pulmonary arteries with donor vessels might be necessary. The timing of transplantation is crucial in this subset of patients; results are better and the choice of donor organs easier whe n growth is almost terminated, in the preadolescent age.