Abstract

Background: Right ventricular (RV) dysfunction is the leading cause of mortality in pulmonary arterial hypertension (PAH), and cardiac magnetic resonance (CMR) measures of RV volumes and mass are known to predict mortality in PAH. Ventricular mass index (VMI), a ratio of RV mass to left ventricular (LV) mass, predicts the presence of PAH in scleroderma. We hypothesized that VMI would predict mortality in incident PAH. Methods: 65 subjects with incident PAH underwent CMR, and VMI was calculated (RV end-diastolic mass/LV end-diastolic mass). Subjects were followed prospectively until death or censor. Associations between VMI and mortality were assessed with Kaplan-Meier (KM) analysis and Cox proportional hazard (CPH) modeling. Results: Subjects were 57±11 years of age; most were women (91%) with connective tissue disease (CTD) (72%) and NYHA functional class I/II symptoms (64%). There was 52% mortality over a median follow up of 8.3 years. KM analysis showed that VMI>0.43, the median for the cohort, was associated with increased risk of death (HR 2.2, 95% CI 1.07-4.46, p Conclusion: VMI, a CMR metric that reflects the effect of PAH on both RV and LV remodeling, most robustly predicts mortality in PAH. Supported by NIH R01HL114910 and U01HL125175

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