Systemic sclerosis & Raynaud's
Cardiac MRI optimizes risk stratification accuracy in connective tissue disease-associated pulmonary arterial hypertension: a retrospective cohort study
Authors' conclusion
Combining RVEF and IVS ECV with COMPERA 2.0 risk assessment model significantly enhances the model's predictive accuracy for clinical deterioration in CTD-PAH patients.
Study context
- Population
- Patients with CTD-associated PAH diagnosed via right heart catheterization, 2017–2023, who underwent cardiac magnetic resonance (CMR) on clinical indicationsmean follow-up 27 months
- Participants
- 110 patients
- Exposure
- COMPERA 2.0 risk assessment model plus CMR metrics vs COMPERA 2.0 alone
- Outcome
- Potential of CMR parameters to augment prognostic evaluation in CTD-PAH
Methodological detailssingle-centre
- Single-centre
Main finding
As reported in the abstract
Prediction of 1-, 2- and 3-year event-free survival, C-index
| Group | Value | C-index |
|---|---|---|
| COMPERA 2.0 | 0.626 | 0.626 |
| COMPERA 2.0 + right ventricular ejection fraction (RVEF) + IVS ECV | 0.805 | 0.805 |
Value
COMPERA 2.00.626
COMPERA 2.0 + right ventricular ejection fraction (RVEF) + IVS ECV0.805
C-index
COMPERA 2.00.626
COMPERA 2.0 + right ventricular ejection fraction (RVEF) + IVS ECV0.805
- Clinical deterioration events
- 27
Observational data: associations, not effects.
Original publication
Cardiac MRI optimizes risk stratification accuracy in connective tissue disease-associated pulmonary arterial hypertension: a retrospective cohort study. Rheumatology (Oxford). Published 11 March 2025. doi:10. 1093/ rheumatology/ keaf140
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