Survival After Lung Transplant for Cystic Fibrosis in Italy: A Single Center Experience With 20 Years of Follow-up

Abstract Objectives Lung transplantationis currently the only treatment for end-stage respiratory failure in patients with cystic fibrosis (CF). In this study we retrospectively analyzed our experience since the start of the transplantation program in 1996 with focus on survival analysis. Methods All patients with CF who underwent lung transplantat our center were included (1996–2016). Survival analysisafter lung transplantwas performed using the Kaplan-Meier estimate, comparing by sex and by 4 eras (1996–2000, 2001–2005, 2006–2010, and 2011–2016). Results In a 20-year period, 243 patients with CF were listed for lung transplant; 123 patients (61 male, 62 female) underwent transplant, and 85 died while waiting for donor organs. The mean (SD) and median age at transplant was 27.7 (8.7) years and 26.9 years (range, 9.1 – 52.1 years), respectively. Mean (SD) forced expiratory volume in the first second was 27.6 (9.7)% predicted; 115 patients (92.0%) were pancreatic insufficient, and 43 patients (34.0%) had CF-related diabetes. Removing patients with CF who died within the first 3 postoperative months, the mean (SD) and median survivalafter transplant were 8.2 (5.7) years and 7.5 years (range, 3 months-20 years), respectively. Overall post– lung transplant1-year survivalwas 93.6%, 5-year survivalwas 71.4%, 10-year survivalwas 53.6%, 15-year survivalwas 36.7%, and 20-year survivalwas 31.6%. We found no difference in survivalbetween sex ( P = .22) and among the 4 eras ( P = .56). Conclusions Survivalafter lung transplantin our single center is similar to international data.