Association and diagnostic utility of diastolic dysfunction and myocardial fibrosis in patients with Fabry disease.

ABSTRACT:

Objectives

Current guidelines highlight important therapy implications of cardiac fibrosis in patients with Fabry disease (FD). However, association between morphological and functional impairments with cardiac fibrosis in hereditary cardiomyopathies remains elusive. We investigated the association between echocardiography-determined cardiac dysfunction and cardiac MRI (cMRI)-detected myocardial fibrosis (late gadolinium enhancement, LE) in patients with FD with preserved left ventricular ejection fraction (≥50%).

Methods

146 patients with FD (aged 39±14 years, 57 men) were analysed, all receiving echocardiography and cMRI within a 1 week interval. Longitudinal systolic strain (LS_sys), strain rate (LSr_sys) and diastolic strain rate (LSr_E/LSr_A) were assessed using speckle

SUBMITTER: Liu D 

PROVIDER: S-EPMC6045729 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature