Lung Transplantation for Cystic Fibrosis

Abstract BACKGROUND The contribution of lung transplantation to the treatment of patients with end-stage cystic fibrosis (CF) has been debated. We aimed to describe achievable outcomes from high-volume CF and lung transplant programs. This study reports on the largest single-center experience of lung transplantation for adult and pediatric CF patients. It also highlights the evolution of practice and outcomes over time. METHODS A retrospective analysis of the prospectively collected Toronto Lung Transplant database was carried out. Post-transplant survival in CF was calculated using the Kaplan-Meier method and analyzed with log-rank tests. RESULTS From 1983 to 2016, 1885 transplants were performed at our institution where 364(19.3%) were CF recipients and another 39(2.1%) were CF re-transplants. The mean age at first transplant was 29.5±9.7y, 56.6% were males, and 91.5% were adults. Pre-transplantation, 88 patients(24.2%) were Burkholderia cepacia complex positive, 143 (39.3%) had diabetes mellitus, and the mean FEV1 was 26.0±7.2% predicted at listing. The 1-, 5-, and 10- probabilities of survival in adults who were BCC negative were 94%, 70%, and 53%, respectively. Pediatric, BCC positive, and re-transplant recipients had worse survival compared to adult BCC negative patients. Strategies to improve the donor pool did not affect survival but possibly reduced waitlist mortality. The most common causes of death after lung transplant for the entire cohort were infection and chronic lung allograft dysfunction. CONCLUSIONS Lung transplantation for CF provides excellent short and long-term outcomes. These results strongly support lung transplantation as the standard of care for CF patients with advanced lung disease.