Predicting Survival in Patients With Pulmonary Arterial Hypertension Raymond L. Benza, MD, Mardi Gomberg-Maitland, MD, C. Greg Elliott, MD, Harrison W. Farber, MD, Aimee J. Foreman, MA, Adaani E. Frost, MD, Michael D. McGoon, MD, David J. Pasta, MS, Mona Selej, MD, Charles D. Burger, MD, Robert P. Frantz, MD  CHEST  DOI: 10.1016/j.chest.2019.02.004 Copyright © 2019 The Authors Terms and Conditions

Figure 1 Strengthening the Reporting of Observational Studies in Epidemiology diagram showing analysis population. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions

Figure 2 A, Comparison of the original REVEAL risk calculator and REVEAL 2.0; (B) overview of modifications in REVEAL 2.0; and (C) REVEAL 2.0 calculator. 6MWD = 6-min walk distance; BNP = brain natriuretic peptide; BPM = beats per min; CTD-PAH = PAH associated with connective tissue disease; Dlco = diffusing capacity of the lungs for carbon monoxide; eGFR = estimated glomerular filtration rate; FC = functional category; HR = heart rate; mRAP = mean right atrial pressure; NT-proBNP = N-terminal fragment of pro-brain natriuretic peptide; NYHA = New York Heart Association; PAH = pulmonary arterial hypertension; PoPH = pulmonary arterial hypertension associated with portopulmonary hypertension; PVR = pulmonary vascular resistance; REVEAL = Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management; SBP = systolic BP; WHO = World Health Organization. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions

Figure 3 Kaplan-Meier survival estimates by Registry to Evaluate Early and Long-Term PAH Disease Management 2.0 risk scores calculated at 1 y postenrollment (baseline for this analysis). A, Twelve-month survival estimate and (B) 60-month survival estimate. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions

Figure 4 Kaplan-Meier estimates of clinical worsening by Registry to Evaluate Early and Long-Term PAH Disease Management 2.0 risk scores calculated at 1 y postenrollment (baseline for this analysis). A, Clinical worsening estimates by all risk scores and (B) clinical worsening among patients with risk scores of 0 to 8. Clinical worsening was defined as all-cause hospitalization or the introduction of a parenteral prostacyclin analog for any reason. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions

Figure 5 Kaplan-Meier survival curves by REVEAL 2.0 (three-category score), COMPERA, and FPHR risk categories from 1 y postenrollment. COMPERA = Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; FPHR = French Pulmonary Hypertension Registry. See Figure 1 legend for expansion of abbreviations. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions

Figure 6 ROC curves and c-statistics at 12 months postbaseline for REVEAL 2.0, REVEAL 2.0 three-category risk score, COMPERA. and FPHR scores. ROC = receiver operating characteristic. See Figure 1 and 5 legends for expansion of other abbreviations. CHEST DOI: (10.1016/j.chest.2019.02.004) Copyright © 2019 The Authors Terms and Conditions