Diffusing Capacity of the Lung for Carbon Monoxide and the Risk of All-Cause Mortality in Patients With Pulmonary Hypertension Caused by Fibrosing Mediastinitis: A Multicenter Cohort Study

Mengyu Wu, Hongling Su, Xuechun Sun, Sugang Gong, Wei Ding, Yuhong Li, Yuting Li, Wenjie Dong, Bowen Jin, Liming Zhao, Qian Jiang, Xincao Tao, Ou Xu, Wande Yu, Jie Cheng, Xintian Cai, Yunshan Cao

https://doi.org/10.1002/pul2.70401 

Abstract

Pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) is a high-risk condition with limited validated tools for prognostic assessment. Although diffusing capacity of the lung for carbon monoxide (DLCO) predicts outcomes in several cardiopulmonary diseases, its prognostic value in PH-FM remains unclear. In this multicenter retrospective cohort study, we included 106 patients with confirmed PH-FM from 14 centers across China. The primary endpoint was all-cause mortality. Cox proportional hazards models were used to assess the association between baseline percent-predicted DLCO and mortality, with sequential adjustment for clinical, hemodynamic, and spirometric variables. The final cohort included 106 patients, of whom 60 had DLCO ≥ 60% predicted and 46 had DLCO < 60% predicted. Over a median follow-up of 36 months (interquartile range [IQR], 14.0–50.8), 27 deaths occurred. Across Cox models, each 10-percentage-point increase in percent-predicted DLCO was associated with a 30% lower risk of all-cause mortality. DLCO < 60% predicted was associated with higher all-cause mortality compared with DLCO ≥ 60% predicted, with hazard ratios ranging from 6.90 to 7.80 across models. Lower baseline percent-predicted DLCO was independently associated with a higher risk of all-cause mortality in patients with PH-FM. DLCO may provide a simple, noninvasive measure to complement current risk assessment in this population.

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