Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in Southern Taiwan: A Single-Center 10-Year Longitudinal Observation Cohort

Author:

Wu Chun-HsinORCID,Lin Chun-YuORCID,Hsu Chih-Hsin,Lin Sheng-Hsiang,Weng Chia-Tse

Abstract

Background: Pulmonary arterial hypertension (PAH) is a life-threatening disease with different etiologies and outcomes. We aimed to explore differences in clinical features and outcomes of idiopathic PAH (iPAH) and connective tissue disease-related PAH (CTD-PAH) in Taiwanese patients and determine risk factors for mortality. Methods: We retrospectively reviewed the medical records of patients with right-sided heart catheterization-diagnosed PAH between January 2005 and December 2015. The iPAH (n = 31) and CTD-PAH (n = 14) patients were enrolled and followed until December 31, 2019. Between-group comparisons were conducted. Potential predictors of the mortality of PAH were determined using the Cox proportional hazard regression model. Results: CTD-PAH patients had higher levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) and lower predicted diffusing capacity of carbon monoxide (DLCO) than iPAH patients. The mortality rates were similar between CTD-PAH and iPAH (21.4% vs. 22.6%, p = 0.99). A mean pulmonary arterial pressure (mPAP) > 46 mmHg was a predictor of PAH-induced mortality (adjusted hazard ratio 21.8, 95% confidence interval 2.32–204.8). Conclusions: A higher mPAP level, but not underlying CTDs, imposed a significantly increased risk of mortality to patients with PAH.

Funder

National Cheng Kung University Hospital

Ministry of Science and Technology

Publisher

MDPI AG

Subject

Health Information Management,Health Informatics,Health Policy,Leadership and Management

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