ORIGINAL RESEARCH article
Front. Med.
Sec. Pulmonary Medicine
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1616252
This article is part of the Research TopicAdvancements in Precision Medicine: Diagnostic and Prognostic Innovations for Cardiopulmonary ConditionsView all articles
Pulmonary Function Impairment and Its Relationship with Target Therapy Response in Patients with Pulmonary Arterial Hypertension
Provisionally accepted
- 1Qilu Hospital, Shandong University, Jinan, China
- 2Xingcheng Community Health Service Center of Xuecheng District, Zaozhuang, Shandong, China
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Introduction: Patients with pulmonary arterial hypertension (PAH) exhibit exertional dyspnea and decreased exercise capacity, which are not solely attributable to right heart dysfunction. Numerous studies have sought to elucidate pulmonary function in PH patients and its correlation with disease severity and prognosis; however, the findings remain inconsistent. The impairment of ventilation and diffusion function may partially account for the occurrence of exertional dyspnea in PAH patients. Methods: This was a single-center prospective observational study. Pulmonary function tests, right heart catheterization and four-strata risk status stratification were performed in PAH patients. The PAH patients were followed up for 12 months. Results: A total of 181 PAH patients were enrolled in the study, comprising 62 with idiopathic pulmonary arterial hypertension (IPAH) and heritable PAH (HPAH), 69 with PAH associated with congenital heart disease (CHD-PAH), and 50 with PAH associated with connective tissue disease (CTD-PAH). The forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), single-breath diffusion capacity for carbon monoxide (DLCO), DLCO% predicted (% pred), and reactance at 5 Hz (X5) were significantly reduced, while residual volume (RV)% pred increased in PAH patients. CHD-PAH exhibited more pronounced ventilation impairment. Six-minute walking distance (6MWD) demonstrated a positive correlation with FEV1 (r=0.353, p <0.01) and FVC (r=0.373, p<0.01), respectively. One-hundred and four patients finished the follow-up. Patients exhibiting FVC% pred values below 82% demonstrated a diminished response to PAH-targeted therapy (OR=10.553, p=0.000, 95% CI: 2.580-43.165). Conclusion: PAH patients exhibited impairment in both ventilation and diffusion capacity, while patients with diverse etiologies demonstrated distinct characteristics. FVC and FEV1 were positively correlated with 6MWD, respectively. PAH patients with FVC% pred values below 82% demonstrated a diminished response to PAH-targeted therapy.
Keywords: pulmonary arterial hypertension, target therapy, Risk status, Pulmonary ventilation function, pulmonary diffusion function
Received: 20 May 2025; Accepted: 22 Sep 2025.
Copyright: © 2025 Xu, Xie, Li, Liu, Yang, Ji, Lu and Cui. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Xia Xu, xuxiaerin@163.com
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