Unique Predictors of Mortality in Patients With Pulmonary Arterial Hypertension Associated With Systemic Sclerosis in the REVEAL Registry

BACKGROUND: Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-APAH) experience higher mortality rates than patients with idiopathic disease and those with other connective tissue diseases (CTD-APAH). We sought to identify unique predictors of mortality associated with SSc-APAH in the CTD-APAH population. METHODS: The Registry to Evaluate Early and Long-Term PAH Management (REVEAL Registry) is a multicenter, prospective US-based registry of patients with previously and newly diagnosed (enrollment within 90 days of diagnostic right-sided heart catheterization) PAH. Cox regression models evaluated all previously identifi ed candidate predictors of mortality in the overall REVEAL Registry population to identify signifi cant predictors of mortality in the SSc-APAH (n 5 500) vs non-SSc-CTD-APAH (n 5 304) populations. RESULTS: Th ree-year survival rates in the previously diagnosed and newly diagnosed SSc-APAH group were 61.4 % 2.7 % and 51.2 % 4.0%, respectively, compared with 80.9 % 2.7 % and 76.4 % 4.6%, respectively, in the non-SSc-CTD-APAH group ( P ,.001). In multi-variate analyses, men aged. 60 years, systolic BP (SBP) 110 mm Hg, 6-min walk distance (6MWD) , 165 m, mean right atrial pressure (mRAP) . 20 mm Hg within 1 year, and pulmo