Congenital heart diseaseCohort study
Haemodynamic Contributors to Pulmonary Hypertension Across the Spectrum of Adult Congenital Heart Disease
- Retrospective cohort study
- single-centre
Authors' conclusion
Elevation of mPAP ≥25 mmHg in ACHD with a biventricular circulation is prognostically important regardless of disease phenotype, but milder PH of 21-25 mmHg is not associated with adverse outcome unless associated with Fontan circulation. Elevation in PVP >15 mmHg and PVR ≥3 Wood units were each individually associated with mortality with combined abnormalities associated with greatest risk.
Study context
- Population
- Adult congenital heart disease (ACHD) patients undergoing cardiac catheterization at Mayo Clinic, 1999–2022
- Participants
- 1,005 patients
- Exposure
- Haemodynamic abnormalities and death/heart transplantation
- Outcome
- Combined endpoint of death/heart transplantation
Main finding
As reported in the abstract
Death/transplant (biventricular circulation)
Hazard ratio (95% CI)P value
PVR ≥3 Wood unitsnot at lower recommended cutpoints
3.00(2.17–4.15)
P < 0.0001mPAP ≥25 mmHgnot at lower recommended cutpoints
3.15(2.17–4.58)
P < 0.0001Combined PVP and PVR abnormalitiesworst outcome
5.20(3.55–7.63)
P < 0.0001Either PVP or PVR abnormalityintermediate risk
2.11(1.46–3.04)
P < 0.0001Observational data: associations, not effects.
Further findings
Prevalence of mean pulmonary artery pressure (mPAP) ≥25 mmHg
- mPAP ≥25 mmHg
- 37%
Fontan (univentricular) circulation
- Only with Fontan circulation was a lower mPAP threshold (20 mmHg) associated with adverse outcomes
Original publication
Haemodynamic Contributors to Pulmonary Hypertension Across the Spectrum of Adult Congenital Heart Disease. Eur J Heart Fail. Published 2 July 2024. doi:10.1002/ejhf.3366
Summarised from the published abstract; every number is checked against it