Heart transplantation in children and infants: Short-term outcome and long-term follow-up

Since 1988, 82 heart transplants have been performed in 80 infants and chil dren. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43), cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6). and other complex congenital heart diseases (n = 12). Age at transplantation wa s < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes or death were: rejection (eight patients): right ventricular failure (four pa tients), transplant coronary artery disease (TCAD) (two patients); and othe r causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two p atients developed post-transplant lymphoproliferative disease, which was tr eated successfully. Major long-term morbidity is neurologic deficit - sever e in three patients and minor in six. TCAD was present or suspected in Six surviving patients. We conclude that heart transplantation in infants and c hildren can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing pro blem in the long term.