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

Description

Calculates the REVEAL 2.0 Risk Score to predict 12-month survival in patients with Pulmonary Arterial Hypertension (PAH). This updated version refines the original REVEAL score by incorporating renal function, all-cause hospitalizations, and modifying cut-points for other variables.

Usage

reveal_pah_risk_score(who_group1_subgroup, age_years, male_sex,
                      renal_insufficiency, nyha_class, systolic_bp, heart_rate,
                      hospitalizations_6mo, six_min_walk_distance,
                      bnp_level = NULL, nt_probnp_level = NULL,
                      pericardial_effusion, dlco_predicted, mrap_gt_20, pvr_lt_5)

Value

A list containing:

REVEAL_2_0_Score: The calculated risk score (Range approx -6 to +23).
Risk_Group: Classification (Low <=6, Intermediate 7-8, High >=9).
Mortality_Risk_12_Month: Estimated 12-month mortality risk.

Arguments

who_group1_subgroup: String. Subgroup of PAH. "portopulmonary": +3 pts. "familial" or "heritable": +2 pts. "ctd" (Connective Tissue Disease): +1 pt. "other" (Idiopathic, Drug/Toxin, CHD, HIV, etc.): 0 pts.
age_years: Numeric. Patient age in years.
male_sex: Numeric (0 or 1). Is the patient male? (1 = Yes). (Male > 60 years adds +2 pts).
renal_insufficiency: Numeric (0 or 1). eGFR < 60 mL/min/1.73m^2 or clinical renal insufficiency. (1 = Yes, +1 pt).
nyha_class: Numeric (1-4). NYHA Functional Class. Class I: -1 pt. Class II: 0 pts. Class III: +1 pt. Class IV: +2 pts.
systolic_bp: Numeric. Systolic blood pressure in mmHg. (< 110 mmHg adds +1 pt).
heart_rate: Numeric. Heart rate in bpm. (> 96 bpm adds +1 pt).
hospitalizations_6mo: Numeric (0 or 1). Any all-cause hospitalizations within the last 6 months? (1 = Yes, +1 pt).
six_min_walk_distance: Numeric. 6-Minute Walk Distance in meters. >= 440m: -2 pts. 320-<440m: -1 pt. 165-<320m: 0 pts. < 165m: +1 pt.
bnp_level: Numeric (Optional). BNP level in pg/mL. <50: -2 pts. 50-<200: 0 pts. 200-<800: +1 pt. >=800: +2 pts.
nt_probnp_level: Numeric (Optional). NT-proBNP level in pg/mL. (If provided, takes precedence or used if BNP missing). <300: -2 pts. 300-<1100: 0 pts. >=1100: +2 pts.
pericardial_effusion: Numeric (0 or 1). Presence of pericardial effusion on echocardiogram. (1 = Yes, +1 pt).
dlco_predicted: Numeric. DLCO percent predicted. (< 40% adds +1 pt).
mrap_gt_20: Numeric (0 or 1). Mean Right Atrial Pressure > 20 mmHg on RHC within 1 year. (1 = Yes, +1 pt).
pvr_lt_5: Numeric (0 or 1). Pulmonary Vascular Resistance < 5 Wood units on RHC. (1 = Yes, -1 pt).

References

Benza RL, Gomberg-Maitland M, Elliott CG, 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

Examples

Run this code


# Example 1: Low Risk
# Idiopathic, 40yo Female, Normal Renal, NYHA II, SBP 120, HR 80,
#No Hosp, 6MWD 450, NT-proBNP 200, No Effusion, DLCO 60, mRAP 10, PVR 4
# Score = 0 + 0 + 0 + 0 + 0 + 0 + 0 - 2 - 2 + 0 + 0 + 0 - 1 = -5
reveal_pah_risk_score("other", 40, 0, 0, 2, 120, 80, 0, 450, NULL, 200, 0, 60, 0, 1)

# Example 2: High Risk
# CTD (+1), 70yo Male (+2), Renal (+1), NYHA III (+1), SBP 100 (+1),
#HR 100 (+1), Hosp (+1), 6MWD 150 (+1), BNP 900 (+2), Effusion (+1),
#DLCO 30 (+1), mRAP 22 (+1), PVR 8 (0)
# Score = 1 + 2 + 1 + 1 + 1 + 1 + 1 + 1 + 2 + 1 + 1 + 1 + 0 = 14
reveal_pah_risk_score("ctd", 70, 1, 1, 3, 100, 100, 1, 150, 900, NULL, 1, 30, 1, 0)

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