Lung transplantation for cystic fibrosis: effective and durable therapy in a high-risk group

Background. The purpose of this study was to review our experience with lung transplantation in patients with end-stage cystic fibrosis. Methods. Eight-two patients with cystic fibrosis have undergone bilateral lung transplantation (n = 76) or bilateral lower lobe transplantation (n = 6) since October 1990. Results. Actuarial survival for the entire cohort is 79% at 1 year and 57% at 5 years. The development of bronchiolitis obliterans syndrome is the leading cause of death after the first year. Freedom from bronchiolitis obliterans syndrome is 84% at 1 year and 51% at 3 years. Pulmonary function tests improve dramatically in recipients. There was no association between death within 1 year and recipient age, weight, graft ischemic time, cytomegalovirus seronegativity, or the presence of panresistant organisms. Similarly, there was no association between the development of bronchiolitis obliterans syndrome within 2 years and ischemic time, number of rejection episodes, cytomegalovirus seronegativity, or the presence of panresistant organisms. Conclusions. Despite their poor nutritional status and the presence of multiply resistant organisms, patients with cystic fibrosis can undergo bilateral lung transplantation with acceptable morbidity and mortality.