PAH Mortality Risk Accurately Predicted by Abridged REVEAL Risk Calculator

pulmonary hypertension , pah, pulmonary arterial hypertension
An abridged version of the REVEAL risk calculator, the REVEAL Lite 2, represents a simpler and accurate tool for predicting mortality risk in patients with pulmonary arterial hypertension.

An abridged version of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator — the REVEAL Lite 2 —represents a simpler and accurate tool for predicting mortality risk in patients with pulmonary arterial hypertension (PAH), according to study results published in CHEST.

The REVEAL Lite 2 incorporates 6 modifiable and noninvasive variables, including functional class (FC), vital signs (systolic blood pressure [SBP] and heart rate), 6-minute walk distance (6MWD), brain natriuretic peptide (BNP)/N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and renal insufficiency (by estimated glomerular filtration rate [eGFR]).

In this study, researchers presented analyses conducted during development as well as internal validation of the REVEAL Lite 2. These analyses included multivariate, Kaplan-Meier, Cox proportional-hazard model, and concordance index analyses.

A total of 2529 patients from the REVEAL registry were eligible for inclusion. Approximately half of the patients had idiopathic PAH and 25% had connective tissue-associated PAH. Almost all (87%) of patients were classified as New York Heart Association (NYHA) FC 2/3.

According to a Cox proportional-hazard multivariable analysis, all variables and scores were considered independent predictors of survival. The most highly predictive variables for survival were BNP/NT-proBNP (chi-squared value for high level=56.4254; P <.0001), followed by 6MWD (chi-squared value for ≥440 m=48.1825; P <.0001), and NYHA/World Health Organization (WHO) FC 4 (chi-squared value=38.0737; P <.0001).

REVEAL Lite 2 still provided good discrimination among risk groups even if several (1-3) less-predictive variables were missing (ie, heart rate, SBP, and eGFR). However, when 2 variables from the top 3 variables were missing (ie, BNP/NT-proBNP, 6MWD, and NYHA/WHO FC), the c-index was <0.70, which the investigators noted was “below the threshold for good discrimination.”

A limitation of this study was the use of a derivative cohort. The study authors noted that a nonderivative cohort is necessary to validate the findings.

The investigators suggested that after the REVEAL Lite 2 is independent validated, the assessment tool “could be used for screening patients with PAH as it readily discriminates between patients at low risk compared with those not at low risk.”

Disclosure: This clinical trial was supported by Actelion Pharmaceuticals. Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.


Benza RL, Kanwar MK, Raina A, et al. Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension. Published online September 1, 2020. CHEST. doi:10.1016/j.chest.2020.08.2069