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Fabry cardiomyopathy presenting with a high defibrillation threshold: A short case report

Takashi Kanda, MD, Masaharu Masuda, MD, Akihiro Sunaga, MD and 8 more

Journal of Arrhythmia · 2015

doi:10.1016/j.joa.2014.10.002 (opens in a new tab)

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Abstract

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.

Abstract licence: CC BY-NC

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