Association of group 3 pulmonary hypertension with clinical outcomes in patients with end-stage non-cystic fibrosis bronchiectasis referred for lung transplantation evaluation

To evaluate the association of Group 3 pulmonary hypertension (PH) with clinical outcomes and established prognostic scoring systems in patients with end-stage non-cystic fibrosis (non-CF) bronchiectasis referred for lung transplantation (LTx) evaluation. This retrospective single-center study included 56 patients with end-stage non-CF bronchiectasis referred for LTx evaluation between January 2017 and September 2024. Disease severity was assessed using the FACED score and the Bronchiectasis Severity Index (BSI). Group 3 PH was defined according to the 2022 European Society of Cardiology/European Respiratory Society guidelines using right heart catheterization findings. Receiver operating characteristic (ROC) analysis was performed to assess the discriminatory performance of the FACED score, BSI, Group 3 PH, and right heart catheterization-derived mean pulmonary arterial pressure (mPAP) for all-cause mortality. Waiting-list survival was evaluated using Kaplan–Meier analysis. Of the 56 patients, 33 (58.9%) were male, and the mean age was 36.4 ± 12.7 years. Group 3 PH was identified in 41 patients (73.2%). During follow-up, 25 patients (44.7%) died. All-cause mortality was significantly higher among patients with Group 3 PH than among those without PH (53.7% vs. 20.0%; p = 0.034). During waiting-list follow-up, 15 patients (26.8%) died before transplantation, 19 (33.9%) underwent transplantation, and 22 (39.3%) remained alive without transplantation. Kaplan–Meier analysis showed lower estimated waiting-list survival in patients with Group 3 PH, although the between-group difference was not statistically significant (log-rank p = 0.294). The FACED score and BSI showed limited discriminatory performance for all-cause mortality, whereas mPAP demonstrated good discriminatory performance (AUC = 0.803, 95% CI 0.665–0.941; p = 0.001). Group 3 PH was associated with higher all-cause mortality in patients with end-stage non-CF bronchiectasis referred for LTx evaluation. Although the FACED score and BSI showed limited prognostic discrimination in this cohort, right heart catheterization-derived mPAP demonstrated better discriminatory performance for all-cause mortality. These findings suggest that hemodynamic assessment may complement established clinical severity scores during LTx evaluation. Larger prospective multicenter studies are needed to validate these findings.