Phenotypic Analysis of Pulmonary Hypertension Associated With Low Diffusion Capacity and Preserved Lung Function.

ABSTRACT: Severe diffusion impairment in pulmonary arterial hypertension (PAH), particularly in idiopathic PAH (IPAH), has garnered considerable attention. However, comprehensive data on low diffusion capacity of the lungs for carbon monoxide (DLCO) with preserved lung function remain limited in broader pulmonary hypertension (PH) cohorts. We analyzed patients with PH, preserved lung function, low DLCO, and available computed tomography (CT) scans. The analysis included 117 patients, with 34% cases of combined pulmonary fibrosis and emphysema (CPFE), 22% IPAH, 15% interstitial lung disease (ILD), 9% pulmonary veno-occlusive disease (PVOD), and 8% connective tissue disease (CTD). Based on hemodynamic and CT imaging features, the overall population could be broadly categorized into two phenotypic patt

SUBMITTER: Zhao QH 

PROVIDER: S-EPMC12616509 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature