Open AccessFabry cardiomyopathy presenting with a high defibrillation threshold: A short case report
Author(s) -
Kanda Takashi,
Masuda Masaharu,
Sunaga Akihiro,
Fujita Masashi,
Iida Osamu,
Okamoto Shin,
Ishihara Takayuki,
Nanto Kiyonori,
Shiraki Tatsuya,
Sera Fusako,
Uematsu Masaaki
Publication year - 2015
Publication title -
journal of arrhythmia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 21
eISSN - 1883-2148
pISSN - 1880-4276
DOI - 10.1016/j.joa.2014.10.002
Subject(s) - medicine , fabry disease , defibrillation , implantable cardioverter defibrillator , cardiology , cardiomyopathy , ventricular fibrillation , enzyme replacement therapy , disease , heart failure
Fabry disease is an X‐linked recessive glycosphingolipid storage disorder caused by a deficiency of lysosomal enzyme α‐galactosidase A. It is recognized that Fabry disease patients often have ventricular arrhythmias. Although the effectiveness of implantable cardioverter‐defibrillator (ICD) therapy in patients with ventricular fibrillation is established, there is little evidence regarding ICD therapy for Fabry disease. Here, we report the case of patient with Fabry disease who was treated with an ICD and presented with high defibrillation thresholds.