Pulmonary vascular disease & thromboembolism
Outcomes in Patients Receiving Treatment for Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease
Authors' conclusion
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.
Study context
- Population
- Patients with pulmonary arterial hypertension, including CHD-PAH patients whose congenital heart disease (CHD) was repaired ≥1 year priorpatients with pulmonary arterial hypertension (PAH), 177 (8.9%) with CHD-PAH · CHD-PAH 177
- Participants
- 1,982 patients
- Comparison
- Approved PAH drug vs placebo
- Outcome
- Time to first confirmed morbidity/mortality (M/M) event
Methodological details3 randomized, placebo-controlled, event-driven studies, random effects models
- 3 randomized, placebo-controlled, event-driven studies
- Random effects models
Pooled result
As reported in the abstract
treatment vs placeboHazard ratio (95% CI)
Overall PAH cohort; 37% reduction
0.63(0.52–0.77)
CHD-PAH population; 50% reduction
0.50(0.26–0.94)
Further pooled results
Baseline characteristics
- Overall PAH cohort mean age, treatment and placebo groups
- 48 and 49 years
- Overall PAH cohort female, treatment and placebo groups
- 80% and 77%
- CHD-PAH cohort mean age
- 41 and 39 years
- CHD-PAH cohort female
- 70% and 66%
- World Health Organization functional class II and III at baseline, in each group
- ≥98%
Original publication
Outcomes in Patients Receiving Treatment for Pulmonary Arterial Hypertension Associated With Repaired Congenital Heart Disease. JACC Adv. Published 24 February 2025. doi:10.
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