The REVEAL (Registry to Evaluate Early and Long-Term PAH Disease Management) 2.0 three category score was better at discriminating risks in patients with pulmonary arterial hypertension (PAH) than similar tools such as COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) and FPHR (French Pulmonary Hypertension Registry), according to a study published in CHEST.

Point values, cut-points, and inclusion of new variables from surviving members with PAH of the US-based registry REVEAL were used to develop the REVEAL 2.0 risk calculator 3-category risk score. The risk discrimination ability of REVEAL 2.0 was compared with the similar risk prediction tools, COMPERA and FPHR.

Of the 3515 patients in the original study ( Identifier: NCT00370214), 2529 were included in the analysis cohort of this study. New variables such as all-cause hospitalization within the previous 6 months and the revised renal insufficiency variable added significantly to the predictive capability of the REVEAL 2.0 calculator when compared with previous versions. Survival rates from 1-year post-enrollment were higher in patients with lower risk scores and lower for those with higher risk scores (P <.001).

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When compared with other predictive tools, COMPERA and FPHR underestimated risk in 80% and 58% of the patients in the highest risk group (>10% 12-month mortality), respectively. Kaplan-Meier survival estimates showed REVEAL 2.0 three-category score provided better characterization of the actual risk for mortality at 12 months than either COMPERA or FPHR.

“Regular and accurate risk assessment affords clinicians a consistent method to follow their patients over time, informs treatment goals and decision making, and assists in the timely referral of patients for transplantation,” the researchers wrote.

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Disclosures: Funding for this analysis was provided by Actelion Pharmaceuticals US, Inc. Several authors report financial relationships with Actelion, Bayer, Gilead, and United Therapeutics, among others.


Benza R, Gomberg-Maitland M, Elliot G, et al. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC/ERS-based risk assessment strategies [published February 14, 2019]. CHEST. doi:10.1016/j.chest.2019.02.004

This article originally appeared on Pulmonology Advisor