133 Left Ventricular Echocardiographic Structural Parameters That Determine Major Adverse Cardiovascular Events (MACE) in Fabry Disease

Abstract

Fabry disease (FD) is an X-linked disorder, with α-galactosidase A deficiency with accumulation of lysosomal glycosphingolipids in multiple organs. Cardiac manifestations, predominantly LV hypertrophy (LVH)1 are common. We evaluated LV structural parameters on transthoracic echocardiographic (TTE) that would predict MACE in FD patients. Retrospective analysis of TTEs for FD patients reviewed at Westmead Hospital Genetics Clinic. MACE was defined as the first adverse event after TTE including death, myocardial infarction, stroke, TIA, exacerbation of heart failure, arrythmia, or implantation of a PPM/AICD. Our cohort consisted of 39 FD patients with mean follow-up of 73 months. MACE occurred in 18/39 patients. Increased indexed LV mass predicted MACE when compared to normal indexed LV mass (17/24 vs 1/15 events, mean time to MACE 79.3 vs 154.7 months, p=0.002). Likewise, increased average wall thickness also predicted MACE when compared to FD patients with normal wall thickness (17/26 vs 1/13 events, mean time to MACE 84.5 vs 152.4 months, p=0.009). Structural parameters including increased LV mass and wall thickness predict MACE in patients with Fabry disease.View Large Image Figure ViewerDownload Hi-res image Download (PPT)