REVEAL 2.0 Clinical Assessment
Validated for WHO Group 1 PAH. Goal-directed therapy targets a Low Risk (≤6) status.
Etiology & History
REVEAL Lite Zone
Clinical & Functional
0
0
Invasive & Diagnostics
DLCO % Predicted
0
Mean RAP (mmHg)
0
PVR (Wood units)
0
Ready for Assessment
Guidelines & Evidence
Verified
Last Review: 2026-07-17
When to Use
Primary Clinical Uses
Predict 1-year and 5-year mortality risk in patients with Pulmonary Arterial Hypertension (PAH)
Guide treatment escalation decisions with goal of achieving or maintaining low-risk status
Monitor treatment response at follow-up assessments (recommended every 3-6 months)
Risk stratify patients with inoperable or persistent/recurrent CTEPH (post hoc validation)
Key Clinical Context
The REVEAL 2.0 score demonstrates greater risk discrimination (c-statistic 0.73-0.76) than COMPERA (0.62) or French registry (0.64) strategies. However, in patients with PH secondary to left heart disease (WHO Group 2), the score accurately predicts high-risk outcomes but may not differentiate intermediate from low risk.
How it Works
Scoring Formula (REVEAL 2.0 — 13 Variables)
Total Score = 6 + Σ(point values from all applicable variables)
PAH Etiology:
• Connective tissue disease (CTD-PAH): +1
• Portopulmonary hypertension (PoPH): +3
• Heritable PAH (HPAH): +2
Demographics:
• Male >60 years: +2
Renal Function:
• eGFR <60 mL/min/1.73m²: +1
NYHA/WHO Functional Class:
• FC I: −1
• FC III: +1
• FC IV: +2
Hospitalization (within 6 months): +1
Vital Signs:
• Systolic BP <110 mmHg: +1
• Heart rate >96 bpm: +1
6-Minute Walk Distance:
• ≥440 m: −2
• 320–439 m: −1
• <165 m: +1
BNP/NT‑proBNP:
• BNP <50 pg/mL OR NT‑proBNP <300 pg/mL: −2
• BNP 200–799 pg/mL: +1
• BNP ≥800 pg/mL OR NT‑proBNP ≥1100 pg/mL: +2
Echocardiogram:
• Pericardial effusion: +1
Pulmonary Function Test:
• DLCO <40% predicted: +1
Hemodynamics (RHC within 1 year):
• Mean RAP >20 mmHg: +1
• PVR <5 Wood units: −1
Risk Stratification (Three‑Category Model)
| Risk Category | Score Range | 1‑Year Mortality | Hazard Ratio (95% CI) |
|---|---|---|---|
| Low Risk | ≤6 | 1.9% (1.1-2.7) | Reference |
| Intermediate Risk | 7–8 | 6.5% (4.7-8.4) | 2.73 (2.2-3.4) |
| High Risk | ≥9 | 25.8% (22.7-28.9) | 8.09 (6.6-9.9) |
REVEAL Lite 2 — Abridged Version (6 Noninvasive Variables)
| Risk Category | Score Range | 1‑Year Mortality | Variables |
|---|---|---|---|
| Low Risk | ≤5 | 2.9% (1.8-3.9) | NYHA/WHO FC, SBP, HR, 6MWD, BNP/NT‑proBNP, eGFR |
| Intermediate Risk | 6–7 | 7.1% (5.4-8.8) | All noninvasive, modifiable |
| High Risk | ≥8 | 25.1% (21.9-28.4) | No invasive hemodynamics required |
Clinical Pearls
Critical Implementation Notes
Minimum 7 variables required for valid REVEAL 2.0 score calculation
Use notation "r" for imaging-based or "p" for pathology-based findings when available
REVEAL Lite 2 is preferred for routine clinical follow-up (no RHC required)
In WHO Group 2 PH (PH-LHD), the score predicts high-risk outcomes but intermediate risk may be overestimated (Sharp-Dimitri et al., 2024)
The TAPSE/sPAP ratio (0.35 mm/mmHg cutoff) improves risk discrimination for scores between 5–8
Performance Metrics
C‑statistic (3‑category): 0.73 (95% CI 0.71-0.75)
C‑statistic (original 8‑tier): 0.76 (95% CI 0.74-0.78)
External validation (PHSANZ Registry): C‑statistic 0.74
REVEAL Lite 2 C‑statistic: 0.70 (95% CI 0.68-0.72)
Next Steps
Treatment Implications (ESC/ERS Guidelines)
01
Low Risk (score ≤6): Continue current therapy; reassess every 6–12 months
02
Intermediate Risk (score 7–8): Consider treatment escalation; reassess every 3–6 months
03
High Risk (score ≥9): Escalate therapy (consider parenteral prostacyclin); refer to expert center
04
REVEAL Lite 2 can be used for more frequent noninvasive monitoring
Special Populations
In CTEPH patients (CHEST study), a 1-point improvement in REVEAL 2.0 was associated with 27% reduction in relative risk of death. In PH-LHD, high-risk patients (score ≥9) had 11-fold increased mortality risk at 3 years (HR 11.10, 95% CI 1.50-81.9).
The Evidence
Primary Sources
Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies
Benza RL et al. • Chest. 2019;Original derivation and validation of REVEAL 2.0 in 2,529 PAH patients. C-statistic 0.76 for 8-tier model, 0.73 for 3-category model.
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
Benza RL et al. • Chest. 2021;Simplified 6-variable noninvasive score. C-statistic 0.70, maintains good discrimination without RHC.
Mortality Risk Assessment Using the REVEAL 2.0 Score in Pulmonary Hypertension Secondary to Left Heart Disease
Sharp-Dimitri D et al. • Research Square. 2024;First validation of REVEAL 2.0 in WHO Group 2 PH. High-risk patients (≥9) had HR 11.10 for 3-year mortality.
Application of the REVEAL risk score calculator 2.0 in the CHEST study
Benza RL et al. • Respiratory Medicine. 2022;Validation in CTEPH patients receiving riociguat. 1-point improvement associated with 27% risk reduction.
Origins & History
REVEAL Registry
The Registry to Evaluate Early and Long‑Term PAH Disease Management (REVEAL) is a multicenter, US‑based observational registry that enrolled 3,515 patients with pulmonary arterial hypertension between 2006 and 2009. The REVEAL 2.0 risk score was published in 2019 as an update to the original 2010 REVEAL risk calculator, incorporating new variables (eGFR, hospitalization within 6 months) and revised cut points to improve risk discrimination.
Last Comprehensive Review: 2026-07-17