Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease
Authors' conclusion
Triple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years.
Study context
- Population
- Patients with PAH associated with congenital heart disease (PAH-CHD)32 with Eisenmenger syndrome
- Participants
- 60 patients
- Exposure
- Triple therapy with prostanoids added to maximal dual combination therapy, before vs after
- Triple therapyn = 60
- Outcome
- 6MWTD, before vs after triple therapy
- Follow-up
- 1 year
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Significant improvement in 6MWTD (before vs 1 year), maintained at 2 years
406 vs 450
p=0.0027
Observational data: associations, not effects.
Further findings
Functional class · 1 and 2 years
- Functional class (FC) improved at 1 year
- 79%
- FC stable at 2 years
- 76%
N-terminal pro-brain natriuretic peptide (NT-proBNP) · 2 years
- Significant decrease; average reduction
- 199 ng/L
Subgroups (Eisenmenger, defect type)
- Greater benefits without Eisenmenger syndrome and with pre-tricuspid defects
Safety
- Patients with side effects, mostly mild and manageable
- 33.3%
Original publication
Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease. Heart. Published 30 October 2023. doi:10.
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