REVEAL_score v.1

REVEAL Registry (Registry for Evaluation of Early and Long-term PAH disease management) Risk Score helps predicting survival in patients with PAH which is important for optimizing therapeutic strategies. REVEAL Score is obtained adding the selected points of the 12 variables + 6. Variables Points WHO group 1 subgroup Associated PAH-connective tissue disease 1 Associated PAH-portopulmonary hypertension 2 Familial PAH 2 Others 0 Renal insufficiency Yes 1 No 0 Male age>60 Yes 2 No 0 NYHA/WHO functional Class I -2 II 0 III 1 IV 2 Systolic BP >=110 mmHg 0 < 110mmHg 1 Heart Rate <=92/min 0 >92/min 1 6-min walk test >=440m -1 165 – 440m 0 <165m 1 BNP, pg/ml <50 -2 50-180 0 >180 1 N-terminal proBNP <300 -2 (BNP not available) 300-1500 0 >1500 1 Pericardial effusion on Yes 1 Echocardiogram No 0 DLCO on Pulmonary >=80% -1 Function Test 33-79% 0 <=32% 1 Mean right atrial pressure Yes 1 >20 mmHg within 1 year No 0 on right heart catheterization Pulmonary vascular resistance Yes 2 >32 Wood units on No 0 right heart catheterization Interpretation of REVEAL Score REVEAL Score Risk Group 1-year predicted survival 0-7 Low >=95% 8 Average 90% - <95% 9 Moderate 85% - <90% 10-11 High 70% - <85% >=12 Very high <70%

Rashmi Damodaran

models@cambiocds.com

@CambioCDS

The purpose is to calculate REVEAL Score and accordingly stratify the patients to different risk groups and predict the 1-year survival in patients with PAH.

It is a simplified calculator used to calculate the REVEAL Score and predict the 1-year survival in patients with PAH.

The REVEAL score can help to decide therapeutic strategies but has not been evaluated as a tool to monitor treatment response

1. Benza RL, Gomberg-Maitland M, Miller DP, Frost A, Frantz RP, Foreman AJ, et al. The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012 Feb;141(2):354–62. 2. Sitbon O, Benza RL, Badesch DB, Barst RJ, Elliott CG, Gressin V, et al. Validation of two predictive models for survival in pulmonary arterial hypertension. European Respiratory Journal. 2015 Jul 1;46(1):152–64. 3. Benza RL, Farber HW, Frost A, Grünig E, Hoeper MM, Busse D, et al. REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat. J Heart Lung Transplant. 2018;37(7):836–43.

openEHR-EHR-OBSERVATION.basic_demographic, openEHR-EHR-OBSERVATION.blood_pressure, openEHR-EHR-OBSERVATION.pulse, openEHR-EHR-OBSERVATION.reveal_registry_risk_score_for_pulmonary_arterial_hypertension, openEHR-EHR-EVALUATION.reveal_registry_risk_score_for_pulmonary_arterial_hypertension