Derivation of a Risk Score (REVEAL-ECHO) Based on Echocardiographic Parameters of Patients with Pulmonary Arterial Hypertension

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Journal Article

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REVEAL; echocardiographic parameters; mortality; outcomes; pulmonary arterial hypertension; risk score


BACKGROUND: Multiparametric risk assessment tools determine mortality risk in patients with pulmonary arterial hypertension (PAH), by combining invasive and noninvasive variables so management strategies can be tailored to individuals. RESEARCH QUESTION: Can a risk score based on common echocardiographic (ECHO) parameters risk stratify PAH patients? STUDY DESIGN AND METHODS: A REVEAL-ECHO risk score was derived using retrospective ECHO data from 2,400 adult PAH patients enrolled in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) database. A stepwise Cox regression model identified ECHO parameters significantly predictive of survival. Values were assigned to each selected parameter based on survival at 12 months' follow-up (Kaplan-Meier estimates). The REVEAL-ECHO risk score was the sum of individual values. Patients were categorized as low, intermediate, or high risk based on Kaplan-Meier predicted 12-month survival. RESULTS: The risk score included four ECHO parameters (right ventricular [RV] chamber enlargement, reduced RV systolic function, tricuspid regurgitation velocity, and pericardial effusion), and accounted for PAH etiology. Higher REVEAL-ECHO risk scores signaled lower probability of 12-month survival. Statistically significant separation of mortality risk was observed among the risk strata: intermediate versus low (hazard ratio [HR], 1.43 [95% confidence interval (CI) 1.17, 1.75, P = .0004]); and high versus low (HR, 2.60 [95% CI 2.19, 3.10, P < .0001]). Augmentation of REVEAL Lite 2 with REVEAL-ECHO achieved separation of REVEAL Lite 2 into four risk groups and identified a subgroup of patients with a low REVEAL Lite 2 risk score who were at higher risk (intermediate-low risk) and a subgroup of patients with an intermediate REVEAL Lite 2 risk score who were also at higher risk (intermediate-high risk) INTERPRETATION: A REVEAL-ECHO risk score, derived using four echocardiographic parameters, may further discriminate risk when used as an adjunct to current risk assessment scores. Further validation is required.