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

Abstract

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