COMBINED LUNG AND LIVER-TRANSPLANTATION IN PATIENTS WITH CYSTIC-FIBROSIS - A 4-1 2-YEAR EXPERIENCE/

Patients with cystic fibrosis who have end-stage respiratory failure a nd associated liver cirrhosis have been considered poor candidates for lung transplantation because of high morbidity and mortality resultin g from hepatic insufficiency after the operation. Since April 1989, ou r policy has been to combine heart-lung or lung and liver transplantat ion in this group of patients. Between June 1990 and March 1995, among 25 patients accepted in the program for combined transplantation, nin e died awaiting transplantation and 10 underwent one of the following procedures: heart-lung-liver transplantation (n = 5), en bloc double l ung-liver transplantation (n = 1), sequential double lung-liver transp lantation (n = 3), and bilateral lobar lung transplantation from a spl it left lung and reduced liver transplantation (n = 1). There were 5 m ale and 5 female patients. The ages of the recipients ranged from 10 t o 24 years. Mean forced expiratory volume in 1 second was 29% and mean forced vital capacity was 35% of predicted values. All patients were infected with resistant Pseudomonas, three with Pseudomonas cepaceia, and two patients had Aspergillus species in addition. All patients had severe cirrhosis with portal hypertension. Four patients had a histor y of esophageal variceal bleeding and two had had previous portosystem ic shunts. The operation was performed as a two-stage procedure, the i ntrathoracic operation being completed before the abdominal stage was begun. Cardiopulmonary bypass was used in all patients because of poor clinical condition. Immunosuppression consisted of azathioprine, cycl osporine, and prednisone, as for isolated lung transplantation. There were two perioperative deaths, one caused by primary liver failure and the second by early lung dysfunction. For the first 3 months after tr ansplantation pulmonary infection was the most common cause of morbidi ty. Other complications included tracheal stenosis (n = 1), bronchial stenosis (n = 1), biliary stricture (n = 2), and severe ascites (n = 3 ). All were successfully treated, Obliterative bronchiolitis developed in three patients. This was stabilized with FK 506 in two patients; t he other patient underwent retransplantation at 38 months but eventual ly died of bleeding. Actuarial survival was 70% at 1 year and remained unchanged at 3 years. Significant functional improvement was observed in all survivors. For patients who have chronic respiratory failure w ith advanced cirrhosis, lung transplant ation combined with liver tran splantation can be performed with a satisfactory outcome.