• James J Anderson, Edmund M Lau, Melanie Lavender, Raymond Benza, David S Celermajer, Nicholas Collins, Carolyn Corrigan, Nathan Dwyer, John Feenstra, Mark Horrigan, Dominic Keating, Fiona Kermeen, Eugene Kotlyar, Tanya McWilliams, Bronwen Rhodes, Peter Steele, Vivek Thakkar, Trevor Williams, Helen Whitford, Kenneth Whyte, Robert Weintraub, Jeremy P Wrobel, Anne Keogh, and Geoff Strange.
• Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; Respiratory Department, Sunshine Coast University Hospital, Birtinya, QLD, Australia. Electronic address: james.anderson2@health.qld.gov.au.
• Chest. 2020 Jan 1; 157 (1): 162-172.

BackgroundPulmonary arterial hypertension (PAH) prognosis has improved with targeted therapies; however, the long-term outlook remains poor. Objective multiparametric risk assessment is recommended to identify patients at risk of early morbidity and mortality, and for optimization of treatment. The US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk score is a new model proposed for the follow-up of patients with PAH but has not been externally validated.MethodsThe REVEAL 2.0 risk score was applied to a mixed prevalent and incident cohort of patients with PAH (n = 1,011) from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) Registry. Kaplan-Meier survival was estimated for each REVEAL 2.0 risk score strata and for a simplified three-category (low, intermediate, and high risk) model. Sensitivity analysis was performed on an incident-only cohort.ResultsThe REVEAL 2.0 model effectively discriminated risk in the large external PHSANZ Registry cohort, with a C statistic of 0.74 (both for full eight-tier and three-category models). When applied to incident cases only, the C statistic was 0.73. The three-category REVEAL 2.0 model demonstrated robust separation of 12- and 60-month survival estimates (all risk category comparisons P < .001). Although the full eight-tier REVEAL 2.0 model separated patients at low, intermediate, and high risk, survival estimates overlapped within some of the intermediate- and high-risk strata.ConclusionsThe 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 patients with PAH at follow-up. The simplified three-category model may be preferred for clinical use and for future comparison with other prognostic models.Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.

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