REVEAL 2.0 is a weighted, additive multivariable model for pulmonary arterial hypertension (WHO Group 1 PAH). The running total starts at 6, then each variable adds or subtracts points (several carry negative points). Enter the most recent clinical, functional, biomarker, echo, and right-heart-catheterization data.
Point assignments:
- Etiology: APAH-CTD +1 · portopulmonary +2 · heritable/familial +1 · other 0
- Renal insufficiency (eGFR <60): +1
- Male >60 years: +2
- WHO Functional Class: I = −1 · II = 0 · III = +1 · IV = +2
- SBP <110 mm Hg: +1 · HR >96 bpm: +1
- 6-minute walk: ≥440 m = −2 · 320–<440 = −1 · 165–<320 = 0 · <165 = +1
- BNP / NT-proBNP: BNP <50 (NT-proBNP <300) = −2 · 50–199 (300–1100) = 0 · 200–800 (>1100) = +1 · >800 = +2
- Pericardial effusion: +1 · DLCO ≤40% predicted: +1
- Mean RAP >20 mm Hg (within 1 yr): +1 · PVR <5 Wood units: −1
Risk groups:
- ≤6: Low risk — predicted 1-year survival >~94%
- 7–8: Intermediate risk
- ≥9: High risk — substantially reduced 1-year survival
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When to Use
Appropriate population
- Confirmed WHO Group 1 PAH — REVEAL 2.0 risk-stratifies established PAH, not pulmonary hypertension from left-heart or lung disease
- Serial follow-up — repeat at each visit; the management goal is achieving and maintaining a low-risk profile
- CKD / dialysis patients — pulmonary hypertension is highly prevalent (~40–60%) in dialysis populations; identifying true Group 1 PAH and stratifying its risk supports cardiology co-management
- Decision points for initiating, escalating, or de-escalating PAH-specific therapy
Pearls & Pitfalls
Key pearls
- The model is additive and weighted, not a simple count — start at 6, then add/subtract each item's points (several are negative)
- Negative-point variables (6MWD ≥440 m, low BNP/NT-proBNP, PVR <5 WU, FC I) reflect favorable findings and pull the score toward low risk
- Cut-points: ≤6 low · 7–8 intermediate · ≥9 high — a falling serial score signals treatment response
- A simplified REVEAL Lite 2 exists — non-invasive, dropping the RAP and PVR (right-heart-catheterization) terms — useful when recent RHC data are unavailable
- Estimate eGFR/CrCl for the renal term; in CKD, kidney impairment legitimately adds risk
- REVEAL 2.0 drives cardiology co-management and the threshold for intensifying PAH-specific therapy
Why Use It
Pulmonary arterial hypertension carries a high mortality, and survival is strongly tied to a small set of clinical, functional, biomarker, and hemodynamic variables. REVEAL 2.0 combines these into a single calibrated risk estimate of 1-year mortality, letting clinicians benchmark a patient against the registry and — by repeating it over time — track whether therapy is moving the patient into the low-risk band. Pulmonary hypertension is common in dialysis populations (~40–60%), so distinguishing and risk-stratifying true Group 1 PAH is clinically relevant in nephrology.
REVEAL 2.0 Risk Calculator
Enter the patient's variables below. The total score and risk group update automatically (the model starts at a base of 6 points).
⚕ Full REVEAL 2.0 model (base score 6 + weighted items). For confirmed WHO Group 1 PAH only; does not apply to PH from left-heart/lung disease. The non-invasive REVEAL Lite 2 omits the RAP and PVR terms. For educational reference; does not replace specialist judgment. Reference: Benza RL et al., Chest 2019.
Next Steps
- Low risk (≤6): Predicted 1-year survival >~94% — continue current PAH-specific therapy and re-score at each follow-up to confirm the patient stays in the low-risk band
- Intermediate risk (7–8): Re-evaluate adequacy of therapy with the cardiology / PH team; consider escalation or add-on combination therapy
- High risk (≥9): Substantially reduced 1-year survival — prompt escalation, consider parenteral prostacyclin and lung-transplant referral where appropriate
- Serial use: A falling score across visits signals treatment response; a rising score warrants intensification
Evidence & References
References
- Benza RL, 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. Chest. 2019;156(2):323–337.
- Benza RL, et al. The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension. Chest. 2012;141(2):354–362.
- Humbert M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618–3731.
