Ratio of Fabry disease in patients with idiopathic left ventricular hypertrophy: A single-center study in Turkey


Barman H. A. , Ozcan S., Atici A., Ozgokce C., Ozturk A., Kafali A. E. , ...More

ANATOLIAN JOURNAL OF CARDIOLOGY, vol.23, no.2, pp.79-85, 2020 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 23 Issue: 2
  • Publication Date: 2020
  • Doi Number: 10.14744/anatoljcardiol.2019.84782
  • Title of Journal : ANATOLIAN JOURNAL OF CARDIOLOGY
  • Page Numbers: pp.79-85

Abstract

Objective: Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism which arises due to deficient or absent activity of lysosomal alpha-galactosidase A (alpha-Gal A). This may be associated with increased left ventricular (LV) wall thickness and may mimic the morphological features of hypertrophic cardiomyopathy. The purpose of this study was to define the ratio of occurrence of FD to the manifestation of unexplained left ventricular hypertrophy (LVH).