Lung pressure predicts death risk in severe bronchiectasis patients awaiting transplants

Patients with end-stage non-cystic fibrosis bronchiectasis who are referred for lung transplantation evaluation face a hidden threat that standard severity scores may miss. A retrospective single-center study of 56 such patients, conducted between January 2017 and September 2024, found that group 3 pulmonary hypertension was present in 41 of them, or 73.2 percent. During follow-up, 25 patients, or 44.7 percent, died, and all-cause mortality was significantly higher among those with group 3 pulmonary hypertension than among those without it, at 53.7 percent versus 20.0 percent, a difference that reached statistical significance with a p value of 0.034.

The study, published in BMC Pulmonary Medicine, set out to evaluate how group 3 pulmonary hypertension relates to clinical outcomes and to established prognostic scoring systems in this population. Disease severity was assessed using the FACED score and the Bronchiectasis Severity Index, while group 3 pulmonary hypertension was defined according to the 2022 European Society of Cardiology and European Respiratory Society guidelines using right heart catheterization findings.

Of the 56 patients, 33, or 58.9 percent, were male, and the mean age was 36.4 plus or minus 12.7 years, indicating a relatively young cohort facing advanced lung disease. During waiting-list follow-up, 15 patients, or 26.8 percent, died before transplantation could occur, 19 patients, or 33.9 percent, underwent transplantation, and 22 patients, or 39.3 percent, remained alive without transplantation. Kaplan-Meier analysis showed lower estimated waiting-list survival in patients with group 3 pulmonary hypertension, although the between-group difference was not statistically significant, with a log-rank p value of 0.294.

The most striking technical finding came from receiver operating characteristic analysis, which assessed how well the FACED score, the Bronchiectasis Severity Index, group 3 pulmonary hypertension status, and catheterization-derived mean pulmonary arterial pressure discriminated between patients who died and those who survived. The FACED score and the Bronchiectasis Severity Index showed limited discriminatory performance for all-cause mortality in this cohort. In contrast, mean pulmonary arterial pressure demonstrated good discriminatory performance, with an area under the curve of 0.803, a 95 percent confidence interval of 0.665 to 0.941, and a p value of 0.001.

In this specific cohort of patients whose lung disease had progressed to the point of transplantation referral, those clinical scores did not separate survivors from nonsurvivors well. The hemodynamic measurement, by directly quantifying pressure in the pulmonary circulation, appeared to capture prognostic information that the clinical scores did not.

The authors conclude that group 3 pulmonary hypertension was associated with higher all-cause mortality in patients with end-stage non-cystic fibrosis bronchiectasis referred for lung transplantation evaluation, and that although the FACED score and the Bronchiectasis Severity Index showed limited prognostic discrimination in this cohort, right heart catheterization-derived mean pulmonary arterial pressure demonstrated better discriminatory performance for all-cause mortality.

The researchers caution that larger prospective multicenter studies are needed to validate these findings. The young mean age of the cohort also distinguishes it from many bronchiectasis populations, and the findings apply specifically to patients with end-stage disease referred for transplantation evaluation rather than to all people with bronchiectasis.

Subject of Research: Group 3 pulmonary hypertension and mortality in end-stage non-cystic fibrosis bronchiectasis patients referred for lung transplantation evaluation

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

Article References: Sarıbaş, E. (2026). Association of group 3 pulmonary hypertension with clinical outcomes in patients with end-stage non-cystic fibrosis bronchiectasis referred for lung transplantation evaluation. BMC Pulmonary Medicine. https://doi.org/10.1186/s12890-026-04961-1

Image Credits: AI Generated

DOI: 10.1186/s12890-026-04961-1

Keywords: pulmonary hypertension, bronchiectasis, lung transplantation, right heart catheterization, mean pulmonary arterial pressure, FACED score, Bronchiectasis Severity Index, all-cause mortality, waiting-list survival, group 3 PH, Association, group