Title: Lung function decline is mitigated following liver transplantation in people with cystic fibrosis: a retrospective cohort study
Source: Liver Transplantation 2023, August 3. [E–publication]
Date of publication: August 2023
Publication type: Retrospective Cohort Study
Abstract: Background: There is paucity of literature on the health outcomes following liver transplantation (LT) in people with cystic fibrosis (pwCF). We aim to evaluate changes in lung function following LT in pwCF.
Methods: We performed a retrospective cohort study of pwCF who underwent LT between 1987 and 2019 in the United States and Canada. Simultaneous lung-liver transplants and individuals who had lung transplant prior to LT were excluded. We analyzed pre- and post-LT percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), rates of pulmonary exacerbation and post-LT overall survival.
Results: A total of 402 LT recipients were included. The median age of transplant was 14.9 years and 69.7% of transplants were performed in children less than 18 years old. The rate of decline in ppFEV1 was attenuated after LT from -2.2% predicted/year to -0.7% predicted/year with a difference of 1.5% predicted/year (95% CI 0.8, 2.2; p < 0.001). Following LT, the rate of decline in BMI was reduced, and there were fewer pulmonary exacerbations (0.6 pre vs. 0.4 post; RR 0.7, p < 0.01). The median survival time post-transplant was 13.9 years and the overall probability of survival at 5 years was 77.6%. Those with higher lung function pre-LT had a lower risk of death post-LT, and those with genotypes other than F508 deletion had worse survival.
Conclusion: LT in pwCF occurs most often in children and adolescents and is associated with a slower rate of decline in lung function and nutritional status, and a reduction in pulmonary exacerbations.