SUCCESSFUL LUNG TRANSPLANTATION IN SPITE OF CYSTIC FIBROSIS-ASSOCIATED LIVER-DISEASE - A CASE SERIES

Lung transplantation has recently offered hope for prolonged survival in patients with cystic fibrosis, Patients with cystic fibrosis have a 7% prevalence of associated liver disease and portal hypertension. Th ese patients have been previously excluded from consideration for lung transplantation, The natural history of cystic fibrosis-associated li ver disease suggests a benign and protracted course in most cases. at the University of Washington, 14 of 53 patients (26%) have undergone l ung transplantation for cystic fibrosis-related respiratory failure. W e report the outcome of double lung transplantation in four of these 1 4 patients who also had cystic fibrosis-associated liver disease and p ortal hypertension, all of whom were symptom free from their liver dis ease. AU four patients are; alive and well without complications 4 to 31 months after transplantation. We conclude that the presence of cyst ic fibrosis-associated IT-ver disease with portal hypertension, in the setting of good synthetic function (albumin > 3.0 gm/L and normal pro thrombin time), normal serum bilirubin, minimal varices, without ascit es or encephalopathy, should not be an absolute contraindication to lu ng transplantation. We recommend that other transplantation centers al so include this patient population in consideration fur lung transplan tation.