Outcomes of Cystic Fibrosis and Non-Cystic Fibrosis Pediatric Lung Transplants: An ISHLT Registry Study

Abstract

Purpose Cystic fibrosis (CF) is a common indication for pediatric lung transplant (LTx), but new CF drug therapies may decrease the need for LTx. Studies of CF vs. non-CF LTx outcomes are rare. We report practice patterns and outcomes for pediatric LTx in CF and non-CF patients. Methods The ISHLT registry was used to report morbidity, graft failure, and survival for primary LTx performed in children ( Results In total, 2,218 primary LTx were performed, 88% in children ≥6yrs. CF was the primary diagnosis in 61%; common non-CF diagnoses were: pulmonary hypertension (28%), pulmonary fibrosis (15%), and obliterative bronchiolitis (12%). CF patients were older (median 15 vs 11yrs, p Conclusion Mid-term mortality and graft failure rates are higher for pediatric LTx recipients with CF, and important gender differences exist. Long-term outcomes are similar between groups. Further study is needed to understand these differences.