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Published on: February 17, 2023
Fabry cardiomyopathy presenting with a high defibrillation threshold: A short case report
Takashi Kanda1, Masaharu Masuda1, Akihiro Sunaga1
1Kansai Rosai Hospital Cardiovascular Center, Amagasaki, Hyogo, Japan 3-1-69 Inabasou, Amagasaki 660-8511, Japan.
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.
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