Progression of left ventricular mass is associated with clinical events in key subgroups of Fabry disease: Analyses from the Fabry Registry

INTRODUCTION: Cardiovascular complications are the primary cause of morbidity and mortality in Fabry disease. Despite disease-specific and adjuvant treatments, gaps in care exist. Cardiac imaging facilitates the understanding of disease progression, however data on the clinical relevance of imaging endpoints in Fabry disease are scant. Therefore, we evaluated the association between left ventricular mass index (LVMi) measured via echocardiography and risk of subsequent clinical events. METHODS: Adult patients from the Fabry Registry treated with enzyme replacement therapy, with echocardiography measurements and no prior kidney events were analyzed. Composite clinical events included cardiac, cerebrovascular, and kidney events, or death. Cox proportional hazard models were used to estimate hazard ratios (HR) and 95 % confidence intervals (CI) for the association between (1) baseline LVMi and (2) LVMi progression (i.e., slopes) with the risk of subsequent clinical events. RESULTS: /year increase in LVMi slope ranged from 1.16 to 1.64 (p < 0.05). CONCLUSIONS: Our study quantifies the biologically plausible association between LVMi and its progression with composite clinical events, including in key subgroups of Fabry patients.