Between May 1988 and June 1989, five combined heart-lung transplants were performed. There were two males, age 16 and 19 years, and three females age 33, 34, and 19 years. Three patients had primary pulmonary hypertension, one had cystic fibrosis, and the fifth had doxorubicin-induced cardiomyopathy with associated pulmonary hypertension. All patients were severely restricted in functional capacity and were oxygen-dependent. Four grafts were distantly procured (ischemic times 1:15, 2:35, 2:45, 3:45); one was procured on-site (ischemic time 0:58). Four of five grafts functioned well (PO2 on FIO2 30% postoperatively: 102, 120, 180, and 129 torr). One graft (distantly procured with an ischemic time of 1:15) showed total failure of oxygenation secondary to fluid overload during donor surgery; the recipient could not be weaned from cardiopulmonary bypass. All patients were extubated 24 to 30 hours postoperatively. Rejection episodes have been infrequent and all have been successfully treated with pulse steroids. Four of the five patients are alive and well with normal cardiopulmonary function and normal functional capacity 15, 12, 8, and 6 months after surgery. Heart-lung transplantation is an effective therapy for properly selected patients with end-stage cardiopulmonary disease resulting from a variety of primary disorders.