Abstract

Aim: Patients with Fabry disease frequently die due to cardiovascular causes. There are numerous findings of cardiovascular involvement in patients with Fabry disease. Epicardial fat thickness is a surrogate for various cardiovascular diseases. In this study, we evaluated epicardial fat thickness and parameters of diastolic dysfunction among Fabry patients as a possible indicator of disease severity.
Materials and Methods: We enrolled 14 patients with Fabry disease who receive enzyme replacement therapy. Detailed echocardiographic examination was performed including diastolic functions and measurement of epicardial fat thickness. Disease severity was assessed using DS3 scoring system. Based on DS3 scores, patients are grouped into mild (

Key words: Fabry’s disease; inflammation; echocardiography.