Key result
Treatment with approved PAH drugs reduced the risk of morbidity and mortality events by 50% (HR 0.50) compared to placebo in patients with pulmonary arterial hypertension associated with repaired congenital heart disease.
Why the study?
Pulmonary arterial hypertension is a common complication in congenital heart disease, but evidence supporting the benefits of approved PAH therapies in repaired CHD-PAH is limited.
Do approved PAH drugs reduce morbidity and mortality events in patients with repaired congenital heart disease-associated PAH?
Meta-Analysis (n=1,982)
Placebo-controlled
Randomized
Yes
Do approved PAH drugs reduce morbidity and mortality events in patients with repaired congenital heart disease-associated PAH?
Approved PAH therapies significantly reduce morbidity and mortality events in patients with repaired congenital heart disease-associated PAH, demonstrating benefits consistent with the overall PAH population.
Answers are grounded in this paper and cite their sources.
Supports similar M/M benefit of PAH therapies in repaired CHD-PAH; leaves open confirmation via dedicated prospective trials.
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 with PAH, 177 (8.9%) with CHD-PAH. In the overall PAH cohort, the mean age was 48 and 49 years in treatment and placebo groups; 80% and 77% were female. In the CHD-PAH cohort, the mean age was 41 and 39 years; 70% and 66% were female. Overall, ≥98% in each group were World Health Organization functional class II and III at baseline. There was a significant reduction in risk of M/M events vs placebo in the overall PAH and CHD-PAH cohorts: 37% reduction in the overall PAH cohort (HR: 0.63; 95% CI: 0.52-0.77) and 50% reduction in the CHD-PAH population (HR: 0.50; 95% CI: 0.26-0.94). CONCLUSIONS: Treatment with approved PAH drugs provided a similar reduction in M/M risk in patients with repaired CHD-PAH when compared with the overall PAH population. This pooled analysis provides important evidence to guide medical management in this patient population.
No takes yet. Share an insight, caveat, or question.
Krasuski et al. (2025) conducted a meta-analysis in Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease (n=1,982). Approved PAH drugs (selexipag, macitentan, bosentan) vs. Placebo was evaluated on Time to first confirmed morbidity/mortality (M/M) event (HR 0.50, 95% CI 0.26-0.94). Treatment with approved PAH drugs reduced the risk of morbidity and mortality events by 50% (HR 0.50) compared to placebo in patients with pulmonary arterial hypertension associated with repaired congenital heart disease.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: