Transplantation for congenital heart disease with special observations on pulmonary atresia and ventricular septal defect

Summary Experience with heart and/or lung transplantation in congenital heart disease has continued to expand during the past decade. While concerns regarding the long-term outlook for transplant recipients remain, it is clear that this therapy can improve survival and quality of life in selected patients with end-stage cardiopulmonary disease when conventional surgical therapy is either not applicable or is unsuccessful. In PA-VSD the option of transplantation is available if severe pulmonary vascular or cardiac anomalies preclude conventional repair and if pulmonary vascular occlusive disease or cardiac failure develops following palliative procedures or total repair. With a number of transplantation options now available, the selection of the optimal procedure for each patient will be dependent on the cardiopulmonary anatomy and physiology.