REVEAL Registry Risk Score 2.0 for Pulmonary Arterial Hypertension (PAH)

REVEAL 2.0 Calculator

WHO Functional Class

Systolic Blood Pressure

Heart Rate

6-Minute Walk Distance

BNP Level

eGFR

DLCO (% predicted)

Pericardial Effusion

Hospitalization for PAH within 6 months

REVEAL 2.0 Score: –

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Predicts survival in patients with pulmonary arterial hypertension.

Why Use

Shown to perform better in the REVEAL cohort than other previously published models, including those developed by Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) registry and French Pulmonary Hypertension Registry (FPHR). Compared to REVEAL 1.0, REVEAL 2.0 has 2 additional/revised inputs, along with revised cutpoints for previously established inputs that improved the predictive power of the tool.

When to Use

Use in patients with recently diagnosed (<3 months ago) WHO Group 1 pulmonary arterial hypertension. Not studied in patients with multiple etiologies of pulmonary hypertension (e.g. Group 1 and Group 3) or pregnant patients; use with caution in these populations. Should not be used to diagnose PAH or determine treatment.

Formula

The REVEAL Score is calculated by the addition of the selected points + 6: Variable Points WHO group 1 subgroup Associated PAH-connective tissue disease 1 Associated PAH-portopulmonary hypertension 3 Familial PAH 2 Other 0 Male age >60 years No 0 Yes 2 eGFR <60 mL/min/1.73 m2 or renal insufficiency (if eGFR is unavailable) No 0 Yes 1 NYHA/WHO functional class I -1 II 0 III 1 IV 2 Systolic BP, mmHg ≥110 mmHg 0 <110 mmHg 1 Heart rate, bpm ≤96 bpm 0 >96 bpm 1 Any hospitalizations within 6 months (for any cause) No 0 Yes 1 6-minute walk test ≥ 440 m -2 320 to <440 m -1 165 to <320 m 0 <165 m 1 BNP*, pg/mL <50 pg/mL -2 50 to <200 pg/mL 0 200 to <800 pg/mL 1 ≥800 pg/mL 2 Pericardial effusion on echocardiogram No 0 Yes 1 DL CO on pulmonary function test ≥40% 0 <40% 1 Mean right atrial pressure >20 mmHg within 1 year on right heart catheterization No 0 Yes 1 Pulmonary vascular resistance <5 Wood units on right heart catheterization Yes -1 No 0 *If N-terminal proBNP is available and BNP is not, listed cut points can be replaced with <300 pg/mL and ≥1,100 pg/mL.

Pearls / Pitfalls

Developed to predict PAH prognosis and objectively monitor treatment response or disease progression. Also used clinically to optimize timing of interventions such as lung transplantation. Only predicts prognosis over a 12 month period. Developed in a US-based registry in which most patients had idiopathic PAH, and may have limited applicability in patients with other etiologies and in other settings.

Management

Patients with PAH WHO Group 1 should be considered for referral to a comprehensive care center for PAH. A multidisciplinary approach should be taken for patients with elevated scores (score >7), including evaluation by a pulmonary hypertension specialist and lung transplant team, along with nutritional assessments, social support, rehabilitation evaluation, and palliative care referral. 2022 ESC/ERC Guidelines recommend that REVEAL scores >10 are appropriate for lung transplant listing if they are undergoing therapy consistent with standard of care. Depending on goals of care and treatment plan, these patients should also be considered for additional clinical evaluation with a six minute walk test and right heart catheterization.

Critical Actions

Hemodynamically unstable or acute right ventricular heart failure with known pulmonary hypertension patients should immediately be hospitalized; consider intensive care monitoring. It remains unknown whether a risk-based, goal-oriented treatment strategy is superior to the standard treatment algorithm recommended in the 2015 ESC/ERS guidelines , which are based on NYHA functional class alone. Risk stratification remains an imperfect science and although REVEAL has respective advantages, it is only a complementary tool for prognostication and guide treatment decisions.

More Information

Interpretation: REVEAL Score Risk Group Predicted 1-Year Survival ≤6 Low ≥94% 7-8 Intermediate 70% to <94% ≥9 High <70%