Abstract
Background: Objective multi-parametric risk assessment is recommended in pulmonary arterial hypertension (PAH) to identify patients at risk of early morbidity and mortality. The REVEAL 2.0 Risk Score is a new model proposed for the follow-up of PAH patients but it has not been externally validated. Methods: The REVEAL 2.0 Risk Score was applied to a mixed prevalent and incident cohort of PAH patients (n=1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier (KM) survival was estimated for each REVEAL 2.0 Risk Score strata and for a broader three-category (low, intermediate, high-risk) model. Results: The REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ registry cohort, with c-statistic 0.74 (both for full 8-tier and three-category models). When applied to incident cases only, c-statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12 and 60-months survival estimates (all risk categories comparisons p Conclusion: The REVEAL 2.0 risk score was validated in a large external cohort from the PHSANZ Registry. The REVEAL 2.0 model can be applied for risk assessment of PAH patients at follow-up. The simplified 3-category model may be preferred for clinical use and for future comparison with other prognostic models.
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