Timely Pulmonary Arterial Hypertension (PAH) Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights
Authors' conclusion
A structured protocol may improve screening for patients with repaired ACHD at risk of developing PAH. RHC may be underutilized in patients with ACHD with TTE findings suggestive of PAH.
Study context
- Population
- Adults ≥18 years with adults with congenital heart disease (ACHD) repaired ≥1 year before enrollment (systemic-to-pulmonary shunt lesions) with additional risk factors for PAH
- Participants
- 217 adults
- Outcome
- PAH screening and detection
- Follow-up
- Maximum 3 years
Methodological detailsstructured protocol, pop-up reminders of evidence-based recommendations, ECG, TTE, WHO functional class and 6-minute walk distance at baseline and yearly for 3 years
- Structured protocol
- Pop-up reminders of evidence-based recommendations
- ECG, TTE, WHO functional class and 6-minute walk distance at baseline and yearly for 3 years
Main finding
As reported in the abstract
maximum follow-up 3 years
- ECG performed annually
- >80%
- TTE performed annually
- >70%
- ≥2 high-risk features for PAH on TTE
- ≈40%
- Underwent right heart catheterization (RHC) among those with high-risk TTE
- 7% (6/82)
- PAH confirmed by RHC
- 2
Observational data: associations, not effects.
Further findings
Baseline characteristics · enrollment
- Mean age, years
- 44.0 ± 15.9
- Women
- 72.3%
- WHO functional class I
- 82.0%
Original publication
Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights. JACC Adv. Published 31 October 2023. doi:10.
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