Long-Term Survival in Pulmonary Arterial Hypertension with Different Etiologies: A Single-Center Experience.

Abstract

Background: Pulmonary arterial hypertension (PAH) is a progressive disease with variable etiologies and poor prognosis. Long-term outcome data across etiologies remain limited.

Methods: We retrospectively studied 70 patients with PAH confirmed by right heart catheterization at Kaohsiung Chang Gung Memorial Hospital (2005-2023). Baseline characteristics, World Health Organization (WHO) functional class (FC), 6-minute walk distance, hemodynamics, comorbidities, and treatment regimens were collected. Survival was analyzed using the Kaplan-Meier method.

Results: The mean age of the patients was 55 years, and 59% were female. Connective tissue disease (CTD) was the leading etiology (35.7%), followed by idiopathic PAH (24.3%) and congenital heart disease (21.4%). The overall survival rates were 80.9%, 76.6%, and 73.4% at 1, 3, and 5 years, respectively. WHO FC IV at diagnosis was significantly associated with worse survival (p < 0.05). Although patients with CTD-PAH had a numerically lower survival rate compared to other etiologies, the difference did not reach statistical significance. Chronic kidney disease (CKD) and coronary artery disease (CAD) were associated with increased mortality. Combination therapy was associated with numerically higher survival (91.8%, 87.0% and 80.8% at 1, 3 and 5 years, respectively) compared to monotherapy or no therapy, although without statistical significance. Baseline risk stratification using the COMPERA 2.0 four-strata model was significantly associated with survival (p < 0.05).

Conclusions: CTD etiology, advanced FC, and comorbid CKD or CAD were associated with a poor prognosis in our cohort of patients with PAH. Combination therapy may improve long-term outcomes.