Survival After Lung Transplant for Cystic Fibrosis in Portugal: A Single Center Experience

<bold>Objectives:</bold> Lung transplantation is 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 Portugal, with a focus on survival analysis. <bold>Methods:</bold> All CF patients who underwent lung transplants at our centre from 2001 to 2023 were included. Survival analysis after lung transplant was performed using the Kaplan-Meier estimate. Cox regression analysis was applied to evaluate the effect of age and gender. <bold>Results:</bold> In the 22 years, 397 patients were submitted to lung transplant, 44 were patients with CF (22 female, 21 male). The mean (SD) age at transplant was 24.4 (±7.8) years. Mean (SD) forced expiratory volume in the first second was 30,8 (±14,4) % predicted; 84% of patients had pancreatic insufficiency and 23% of patients had CF-related diabetes. The mean survival time after transplant was 13.6 (range, 11 – 16.3) years. Overall post-lung transplant 1-year survival was 87%, 5-year survival was 67% and 10-year survival was 67%. We found no difference in survival between gender (p = 0.372) or age (p = 0.435). <bold>Conclusion:</bold> Our study highlights the importance of lung transplantation for patients with CF who are in end-stage respiratory failure. The study findings demonstrate a substantial proportion of patients, 70,7%, still alive at the time of analysis. The inability to calculate a median survival time underscores the longevity achieved by many recipients, emphasizing the need for continued follow-up to fully appreciate the long-term outcomes of lung transplantation in this patient population.