Performance of the REVEAL model in WHO Group 2 to 5 pulmonary hypertension: Application beyond pulmonary arterial hypertension

Background: The majority of patients with pulmonary hypertension (PH) have non-pulmonary arterial hypertension PH (non-PAH PH) or multifactorial PH. The REVEAL score was designed to predict 1-year survival in patients with pulmonary arterial hypertension (PAH) only. It is unknown whether this model is applicable to a more general population of PH patients. Methods: Both newly diagnosed and previously diagnosed patients with PH of any etiology (n = 200) were enrolled in an observational cohort between the years 2003 and 2009. REVEAL scores were assessed for the ability to predict 1-year survival in the following groups: (1) PAH; (2) non-PAH PH; (3) multifactorial PH; and (4) the entire cohort. Results: Of the 200 patients, 126 (63%) had PAH, 32 (16%) had non-PAH PH and 42 (21%) had multifactorial PH. The concordance indices for the model when applied to the various groups were: PAH, 0.72; non-PAH PH, 0.97; multifactorial PH, 0.77; and entire cohort, 0.775. Observed and predicted survivals of the entire cohort according to model-assigned risk strata were not statistically different from one another (p = 0.60), suggesting adequate model calibration. Conclusions: The REVEAL survival prediction model for PAH has comparable performance when applied to a broad population of PH patients. The data suggest that the model may have utility in PH patients in general, subject to validation in a larger cohort.