REVEAL 2.0 risk score: how the PAH points total works

The REVEAL 2.0 risk score for pulmonary arterial hypertension (PAH) starts at 6 and adds or subtracts points for each published finding. A total of 0 to 6 is low risk, 7 to 8 is intermediate and 9 or more is high, for death within 12 months.

Not a medical device. Not a diagnosis or dosing recommendation.

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Respiratory calculators
Benza RL et al. · 2019
The REVEAL 2.0 formula
Worked examples
Frequently asked questions

What is the REVEAL 2.0 risk score?

REVEAL is the Registry to Evaluate Early and Long-Term PAH Disease Management, a US registry. Benza and colleagues published the updated 2.0 calculator in Chest in 2019. It's a prognostic score: findings that point to better function subtract points, and findings that point to worse function add them.

Magent's calculator returns the total, the maximum of 23 and the risk band: low, intermediate or high.

This page explains the arithmetic. It doesn't say what a score means for a particular person.

The REVEAL 2.0 formula

REVEAL 2.0 = 6 (starting points)
  + PAH subgroup: connective tissue disease +1 · portopulmonary +3 · heritable +2 · other 0
  + male and older than 60 +2
  + renal insufficiency +1
  + WHO functional class: I -1 · II 0 · III +1 · IV +2
  + hospitalized in the prior 6 months +1
  + systolic pressure < 110 mm Hg +1
  + heart rate > 96 bpm +1
  + 6-minute walk: ≥ 440 m -2 · 320 to < 440 -1 · 165 to < 320 0 · < 165 +1
  + BNP: < 50 -2 · 50 to < 200 0 · 200 to < 800 +1 · ≥ 800 +2
  + pericardial effusion +1
  + DLCO < 40% predicted +1
  + mean right atrial pressure > 20 mm Hg +1
  + PVR < 5 Wood units -1

low 0 to 6 · intermediate 7 to 8 · high ≥ 9 · maximum 23

Exact expression the calculator uses:

REVEAL 2.0 = 6 + etiology + male_gt_60 + renal_insufficiency + who_fc + hospitalization_6mo + sbp_lt_110 + hr_gt_96 + six_minute_walk + bnp_or_nt_probnp + pericardial_effusion + dlco_lt_40 + mrap_gt_20 + pvr_lt_5; etiology ctd +1, poph +3, heritable +2, other 0; male and age >60 +2; eGFR <60 or renal insufficiency +1; WHO FC I -1, II 0, III +1, IV +2; all-cause hospitalization in prior 6 months +1; SBP <110 mmHg +1; HR >96 bpm +1; 6MWD >=440 m -2, 320-<440 m -1, 165-<320 m 0, <165 m +1; BNP <50 or NT-proBNP <300 -2, BNP 50-<200 or NT-proBNP 300-<1100 0, BNP 200-<800 +1, BNP >=800 or NT-proBNP >=1100 +2; pericardial effusion +1; DLCO <40% predicted +1; mRAP >20 mmHg +1; PVR <5 WU -1; low 0-6, intermediate 7-8, high >=9 (12-month mortality); max 23

Inputs

Input	Meaning	Accepted values
`who_fc`	WHO functional class	1 to 4
`etiology`	PAH subgroup	`other`, `ctd`, `poph`, `heritable`
`age`, `male`	Age in years and male sex	age 18 to 120
`renal_insufficiency`	eGFR under 60 or renal insufficiency	yes or no
`hospitalization_6mo`	Any hospital stay in the prior 6 months	yes or no
`sbp_lt_110`, `hr_gt_96`	Systolic under 110 mm Hg; heart rate over 96 bpm	yes or no
`six_minute_walk_m`	6-minute walk distance, m	0 to 1000
`bnp_pg_ml`	BNP, pg/mL	0 to 35000
`nt_probnp_pg_ml`	Or NT-proBNP, pg/mL, when BNP isn't sent	0 to 35000
`pericardial_effusion`, `dlco_lt_40`, `mrap_gt_20`, `pvr_lt_5`	Echo, lung function and catheter findings	yes or no

How the calculation works

Start at 6.
Add points for the PAH subgroup, for being male and older than 60, and for each yes/no finding that is present.
Functional class: class I subtracts 1, class II adds nothing, class III adds 1, class IV adds 2.
Walk distance moves the total from -2 to +1 and BNP from -2 to +2, by band.
PVR under 5 Wood units subtracts 1.
Band the total: 0 to 6 low, 7 to 8 intermediate, 9 or more high.

Units

BNP and NT-proBNP are in pg/mL and have different cut-offs. If both are sent, BNP is used. Walk distance is in meters. A missing value, one outside its range or a functional class other than 1 to 4 is rejected with an error.

Worked examples

These use Magent's own expected values for the calculator. Each is a 40-year-old woman with the subgroup set to other and no yes/no findings unless stated.

Example 1: the lowest-risk combination

Functional class I, walk 480 m, BNP 40 pg/mL, PVR under 5 Wood units.

Start 6. Class I -1. Walk 480 m -2. BNP 40 -2. PVR -1.
Total 0 → low.

Example 2: functional class III

Functional class III, walk 200 m, BNP 100 pg/mL.

Start 6. Class III +1. Walk 200 m and BNP 100 both fall in 0-point bands.
Total 7 → intermediate.

Example 3: class IV with a recent hospital stay and low blood pressure

Functional class IV, hospitalized in the prior 6 months, systolic under 110, walk 200 m, BNP 100 pg/mL.

Start 6. Class IV +2. Hospital stay +1. Systolic under 110 +1.
Total 10 → high.

History of the REVEAL 2.0 score

The calculator cites Benza and colleagues, Chest, 2019 (volume 156, issue 2, pages 323–337). The abstract describes an update of the REVEAL registry calculator in patients who had survived at least 1 year after enrollment. Point values and cut-points were reassessed and new variables tested, survival at 12 months was estimated with the Kaplan-Meier method, and the result was compared with European guideline-based risk strategies.

When it's used

The score is used in adults with established pulmonary arterial hypertension, from findings gathered at a follow-up assessment.

Where it's used

The score draws on functional class, vital signs, a 6-minute walk test, a BNP or NT-proBNP result, an echocardiogram, lung function testing and right heart catheter measurements.

Who uses it

The person using the calculator enters each finding. The calculator adds the points and assigns the band.

Limits of the calculation

The calculator doesn't time the walk, measure BNP, read the echocardiogram or perform a catheter study. Every finding comes from the person entering it.
It's REVEAL 2.0. It isn't the earlier REVEAL calculator or REVEAL Lite 2.
The bands describe 12-month mortality groups. They are prognostic and don't stand in for a care plan.
This page doesn't say what to do with a score.

Clinical decisions belong to a qualified clinician who knows the patient.

6-minute walk distance gives a predicted walk distance. REVEAL 2.0 uses the measured distance as one of its items.
NIVO score is a separate respiratory points score with its own inputs.

Glossary

WHO functional class: a grading of how much PAH limits daily activity, from I to IV.
BNP / NT-proBNP: blood markers released by a stretched heart, in pg/mL.
DLCO: diffusing capacity of the lung for carbon monoxide, as a percent of predicted.
PVR: pulmonary vascular resistance, in Wood units.
mRAP: mean right atrial pressure, in mm Hg.

Common mistakes

Forgetting the starting 6. Every total begins at 6.
Using BNP cut-offs for NT-proBNP. They have separate bands.
Scoring male sex at any age. The 2 points apply when the patient is male and older than 60.
Using the earlier REVEAL cut-offs. The heart rate cut-off differs in version 2.0.

Source

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. DOI: 10.1016/j.chest.2019.02.004. PMID: 30772387

Frequently asked questions

How is the REVEAL 2.0 score calculated?

Start at 6, then add or subtract the points for each finding.

What REVEAL 2.0 score is high risk?

9 or more. 7 to 8 is intermediate and 0 to 6 is low.

Can I use NT-proBNP instead of BNP?

Yes, when BNP isn't sent. NT-proBNP has its own bands, and BNP is used if both are sent.

Why can the score go below 6?

Good functional class, a long walk distance, a low BNP and a low PVR subtract points.

Is this the same as REVEAL Lite 2?

No. This is the full REVEAL 2.0 score.