Systemic sclerosis & Raynaud's

Cardiac MRI optimizes risk stratification accuracy in connective tissue disease-associated pulmonary arterial hypertension: a retrospective cohort study

Rheumatology (Oxford)Published 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
GroupValueC-index
COMPERA 2.00.6260.626
COMPERA 2.0 + right ventricular ejection fraction (RVEF) + IVS ECV0.8050.805

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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