Volume 141, Issue 2, Pages 354-362 (February 2012) The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial Hypertension  Raymond L. Benza, MD, Mardi Gomberg-Maitland, MD, FCCP, Dave P. Miller, MS, Adaani Frost, MD, FCCP, Robert P. Frantz, MD, Aimee J. Foreman, MA, David B. Badesch, MD, FCCP, Michael D. McGoon, MD, FCCP  CHEST  Volume 141, Issue 2, Pages 354-362 (February 2012) DOI: 10.1378/chest.11-0676 Copyright © 2012 The American College of Chest Physicians Terms and Conditions

Figure 1 REVEAL Registry PAH risk score calculator. Calculated risk scores can range from 0 (lowest risk) to 22 (highest risk). If N-terminal proBNP is available and BNP is not, listed cut points are replaced with < 300 pg/mL and > 1500 pg/mL. APAH = associated pulmonary arterial hypertension; BNP = brain natriuretic peptide; BPM = beats per minute; CTD = connective tissue disease; DLco = diffusing capacity of lung for carbon monoxide; FPAH = familial pulmonary arterial hypertension; HR = heart rate; mRAP = mean right atrial pressure; NYHA = New York Heart Association; PAH = pulmonary arterial hypertension; PoPH = portopulmonary hypertension; PVR = pulmonary vascular resistance; REVEAL Registry = Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management; SBP = systolic BP; WHO = World Health Organization. CHEST 2012 141, 354-362DOI: (10.1378/chest.11-0676) Copyright © 2012 The American College of Chest Physicians Terms and Conditions

Figure 2 Twelve-month Kaplan-Meier survival estimate for the REVEAL Registry development cohort with predicted risk score. Risk strata are indicated by the lines: predicted 1-year survival is 95% to 100% in the low-risk group, 90% to < 95% in the average-risk group, 85% to < 90% in the moderately high-risk group, 70% to < 85% in the high-risk group, and < 70% in the very high-risk group. See Figure 1 legend for expansion of abbreviation. CHEST 2012 141, 354-362DOI: (10.1378/chest.11-0676) Copyright © 2012 The American College of Chest Physicians Terms and Conditions

Figure 3 A, One-year survival in the validation cohort stratified according to estimated probability of surviving 1 year. Predicted 1-year survival is 95% to 100% in the low-risk group, 90% to < 95% in the average-risk group, 85% to < 90% in the moderately high-risk group, 70% to < 85% in the high-risk group, and < 70% in the very high-risk group. B, One-year survival in the validation cohort stratified according to risk score. The average predicted 1-year survival is 95% to 100% (low risk) for patients with risk scores of 1 to 7. Similarly, the ranges specified for average risk, moderately high risk, high risk, and very high risk correspond to risk scores of 8, 9, 10 to 11, and ≥ 12. CHEST 2012 141, 354-362DOI: (10.1378/chest.11-0676) Copyright © 2012 The American College of Chest Physicians Terms and Conditions

Figure 4 Model calibration plots. A, Observed 1-year Kaplan-Meier estimates vs 1-year survival predicted by the prognostic equation. The Xs correspond to the observed survival in the five risk strata based on the prognostic equation. Predicted 1-year survival is 95% to 100% in the low-risk group, 90% to < 95% in the average-risk group, 85% to < 90% in the moderately high-risk group, 70% to < 85% in the high-risk group, and < 70% in the very high-risk group. B, Xs correspond to the observed survival in the five risk strata based on the risk score. Risk score ranges are 1 to 7 in the low-risk group, 8 in the average-risk group, 9 in the moderately high-risk group, 10 to 11 in the high-risk group, and ≥ 12 in the very high-risk group. CHEST 2012 141, 354-362DOI: (10.1378/chest.11-0676) Copyright © 2012 The American College of Chest Physicians Terms and Conditions