Cardiothoracic transplantation for congenital heart disease in teenagers and adults

Abstract

Since 1980, 194 patients aged between 10–52 years have undergone cardiothoracic transplantation for congenital heart disease (CHD) at our institution. Sixty underwent heart transplantation, 129 heart-lung transplantation, and 5 bilateral lung transplantation with repair of the underlying defect. This represented 4%, 27% and 3 % of all patients undergoing heart, heart-lung and bilateral lung transplantation respectively. Patients undergoing heart transplantation either had complex abnormalities with low pulmonary artery pressure (n = 37) or myocardial failure after previous reparative operations (n = 23). Fourteen patients undergoing heart-lung transplantation had complex pulmonary atresia and 115 had CHD with pulmonary hypertension. The type of operation has been modified during the period under review to take account of the increasing scarcity of donor organs. Operative techniques are adapted according to the complexity of the underlying anatomy. In-hospital mortality was 20/60 (33%) for hearts, 41/115 (36%) in heart-lung transplantation for CHD and pulmonary hypertension and 12/14 (85%) for complex pulmonary atresia. There were no deaths in the five patients undergoing bilateral lung transplantation and repair. Major risk factors for early death in the heart-lung transplant group were complex pulmonary atresia, previous lateral thoracotomy and age greater than 35 years at transplant. Actuarial survival in the heart transplants was 60%, 54% and 46% at 1 year, 5 years, and 10 years respectively and 61%, 45% and 36% at 1 year, 5 years and 10 years respectively for heart-lung transplant patients with CHD and pulmonary hypertension. It is concluded that risk of early death is greater in patients with complex CHD undergoing heart transplantation than in patients with acquired heart disease. Thereafter survival in patients with CHD is similar to other patients undergoing heart or heart-lung transplantation.