Outcomes in Patients Receiving Treatment for Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease.

ABSTRACT:

Background

Pulmonary arterial hypertension (PAH) is a common complication among patients with congenital heart disease (CHD). Despite advances in PAH treatment, evidence for the benefits of PAH therapies in CHD-PAH is limited.

Objectives

This analysis aimed to evaluate outcomes in patients with repaired PAH-CHD receiving an approved PAH drug.

Methods

This was a pooled analysis including CHD-PAH patients whose CHD was repaired ≥1 year prior from 3 randomized, placebo-controlled, event-driven studies: GRIPHON (NCT01106014), SERAPHIN (NCT00660179), and COMPASS-2 (NCT00303459). The primary endpoint was time to first confirmed morbidity/mortality (M/M) event. HRs with 95% CIs were determined with random effects models.

Results

The analysis included 1,982 patients wi

SUBMITTER: Krasuski RA 

PROVIDER: S-EPMC11908551 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature