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

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 r...

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Bibliographic Details
Published in:Healthcare (Basel) 2021-05, Vol.9 (5), p.615
Main Authors: Wu, Chun-Hsin, Lin, Chun-Yu, Hsu, Chih-Hsin, Lin, Sheng-Hsiang, Weng, Chia-Tse
Format: Article
Language:English
Subjects:
Antigens
Congenital diseases
connective tissue disease
Connective tissue diseases
HIV
Human immunodeficiency virus
Lupus
mean pulmonary arterial pressure
Medical prognosis
Mortality
Patients
Peptides
pulmonary arterial hypertension
Pulmonary arteries
Pulmonary hypertension
Rheumatology
Survival analysis
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Summary: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.
ISSN:2227-9032
2227-9032
DOI:10.3390/healthcare9050615