Abstract
Fabry disease (FD) is an X-linked lysosomal storage disorder in which glycosphingolipids accumulate in tissues, causing multisystem disease. FD is associated with mild tricuspid, aortic, and mitral valvular heart disease (VHD).1-3 The burden of VHD in the aging FD population treated with modern medical therapy, including enzyme replacement therapy (ERT), substrate reduction therapy, and aggressive control of cardiovascular comorbidities, is unknown. We describe the burden of VHD in a multicenter cohort of patients with FD with access to contemporary FD therapy.
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