Heart-lung transplantation for complex pulmonary atresia: a single centre experience

Abstract

Since 1985, 151 heart-lung transplants have been performed at Harefield Hospital for congenital heart disease. Twenty (15%) of these patients had complex pulmonary atresia (PA) with a ventricular septal defect and major arterio-pulmonary collateral arteries and constitute the subject of this abstract. The ages of the patients were between 1.1 and 43 (mean 18.3) years. There were 13 males and 7 females. In 6 patients there had been no previous operation, 7 patients had undergone 1 lateral thoracotomy, 4 patients 2 lateral thoracotomies and 3 patients 3 or more lateral thoracotomies. Sixteen patients (80%) died without leaving hospital after their transplant (0–130 days). Two of the younger patients died of rejection, 1 of which was hyperacute, and 1 child died of graft failure. The remaining 13 patients died as a consequence of bleeding and massive transfusion, which led to multi-organ failure. There was 1 survivor from the group of patients who had had no previous operations and 3 from the group that had had 1 previous thoracotomy. Factors contributing to bleeding included extensive native mediastinal arterial and venous collaterals, adhesions from previous operations and hyperactive sternal bone marrow secondary to polycythaemia. There were 2 late deaths, both from obliterative bronchiolitis, 2.4 and 7.5 years after transplantation (Tx). Two patients (10%) are alive and well 12.5 and 17.5 years after Tx. Fourteen of the 16 early deaths occurred prior to 1992, when our policy for acceptance for Tx of complex PA was reviewed. Since that date 4 patients referred for Tx underwent a palliative procedure with 1 death, 7 further patients underwent stage correction with 1 death (14%). Five patients were either removed from or died on the waiting list, 2 patients were transplanted and both died. One adult patient remains on the waiting list and no new patient has been added to it since 1998. It is concluded that heart-lung Tx in this group of patients has very poor results. These results have led to a policy in our unit of only accepting such patients under very exceptional circumstances.