Key result
Initial PAH-targeted therapy was associated with a 15-year survival rate of 86.8% compared to 59.4% with conservative treatment, with the most significant prognostic benefit observed in patients with idiopathic PAH.
Why the study?
The advent of targeted therapy transformed pulmonary arterial hypertension treatment in Korea, prompting an analysis of baseline attributes, treatment trends, and survival shifts.
Does early initiation of PAH-targeted therapy improve survival in patients with pulmonary arterial hypertension?
Observational (n=230)
No
Does early initiation of PAH-targeted therapy improve survival in patients with pulmonary arterial hypertension?
The transition towards early initiation of PAH-targeted therapy significantly improved survival rates, particularly in patients with idiopathic pulmonary arterial hypertension.
Supports considering immediate targeted therapy after PAH diagnosis, especially IPAH; leaves open causality and generalizability from this observational cohort.
BACKGROUND AND OBJECTIVES: The transformation of pulmonary arterial hypertension (PAH) treatment in Korea, ushered by targeted therapy's advent, prompted our analysis of baseline attributes, treatment trends, and survival shifts within our single-center registry. METHODS: We examined 230 patients (72.6% female, mean age 40.6±17.4 years) diagnosed and/or treated between 1980 and 2021 in our PAH clinic. Given targeted therapy's introduction and active use since 2007, we compared diagnostic classification, demographics, and treatment patterns at that juncture. Survival analysis encompassed PAH types and the overall population. For historical survival comparison, 50 non-registry patients were retrospectively added, and age-sex matching enabled pooled analysis. RESULTS: Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) constituted the largest subset (43.0%), trailed by connective tissue disease-associated PAH (CTD-PAH, 29.6%) and idiopathic PAH (IPAH, 19.1%). Post-2007, CTD-PAH proportions surged, notably with an elevated initiation rate of targeted therapy (95.4%). Overall survival rates at 1, 5, and 10 years stood at 91.3%, 77.4%, and 65.8%, respectively, with CHD-PAH exhibiting superior survival to idiopathic or CTD-PAH. Age-sex matching analysis indicated survival disparities between those starting immediate targeted therapy vs. conservative treatment upon diagnosis, especially driven by IPAH. CONCLUSIONS: In the post-introduction of the targeted therapy era, patients with PAH promptly started treatment right away, and higher survival rates of patients who started initial PAH-targeted therapy were demonstrated. The transition towards early treatment initiation might have likely contributed to the elevated survival rates observed in Korea's PAH patient cohort.
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Jang et al. (2024) conducted an observational in Pulmonary Arterial Hypertension (n=230). Initial PAH-targeted therapy vs. Initial conservative treatment was evaluated on 15-year overall survival (HR 0.63, 95% CI 0.39-1.01, p=0.06). Initial PAH-targeted therapy was associated with a 15-year survival rate of 86.8% compared to 59.4% with conservative treatment, with the most significant prognostic benefit observed in patients with idiopathic PAH.
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