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

Barman, Hasan; Ozcan, Sevgi; Atici, Adem; Ozgokce, Caner; Ozturk, Ahmet; Kafali, Aysegul; Cakar, Nafiye; Tavsanli, Mustafa; Kucuk, Mehmet; Sabin, Irfan; Okuyan, Ertugrul

doi:10.14744/anatoljcardiol.2019.84782

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

Atıf İçin Kopyala

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

ANATOLIAN JOURNAL OF CARDIOLOGY, cilt.23, sa.2, ss.79-85, 2020 (SCI İndekslerine Giren Dergi)

Cilt numarası: 23 Konu: 2
Basım Tarihi: 2020
Doi Numarası: 10.14744/anatoljcardiol.2019.84782
Dergi Adı: ANATOLIAN JOURNAL OF CARDIOLOGY
Sayfa Sayıları: ss.79-85

Özet

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).