Pulmonary Hypertension Prevalence and Significance in Lung Transplant Recipients With Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

Background: Pulmonary hypertension (PH) is a complication of advanced cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis (n-CFB). The prevalence and posttransplantation prognostic significance of PH in CF and n-CFB remain poorly defined. Research Question: What is the prevalence of PH in patients with CF and n-CFB listed for lung transplantation, and how does pretransplantation PH affect posttransplantation survival in each group? Study Design and Methods: Patients with CF or n-CFB listed for lung transplantation with right heart catheterization data between 2013 and 2024 were included. PH was defined per the 7th World Symposium on Pulmonary Hypertension criteria. Survival was analyzed using Fine-Gray models with time-varying covariates to account for nonproportional hazards and competing risks. Results: The cohort included 1,273 transplant recipients with CF and 377 with n-CFB with pretransplantation right heart catheterization data. PH was frequently observed in both conditions, although it was more prevalent in CF than n-CFB (60% vs 46%; P <. 001). Among patients with CF, PH was associated with lower early posttransplantation mortality (subdistribution hazard ratio [sHR], 0.7; 95% CI, 0.5-0.9) but with an increased hazard over time (interaction sHR, 1.4; 95% CI, 1.1-1.9), with worse outcomes beyond 3 years following transplantation. In contrast, PH was not significantly associated with posttransplantation survival among patients with n-CFB (sHR, 0.8; 95% CI, 0.5-1.3). Interpretation: Our results show that precapillary PH is common and potentially underappreciated in patients with advanced CF and n-CFB listed for lung transplantation, with a higher prevalence of PH observed in CF. In this CF cohort, PH appears to be paradoxically associated with improved early posttransplantation survival but is followed by diminished survival over time. These findings highlight a complex and time-dependent relationship between PH and transplantation outcomes in CF, warranting further investigation, particularly in the context of cystic fibrosis transmembrane conductance regulator modulator therapy.