Journal of Arrhythmia (Jun 2015)

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

  • Takashi Kanda, MD,
  • Masaharu Masuda, MD,
  • Akihiro Sunaga, MD,
  • Masashi Fujita, MD, PhD,
  • Osamu Iida, MD,
  • Shin Okamoto, MD,
  • Takayuki Ishihara, MD,
  • Kiyonori Nanto, MD,
  • Tatsuya Shiraki, MD,
  • Fusako Sera, MD,
  • Masaaki Uematsu, MD, PhD

DOI
https://doi.org/10.1016/j.joa.2014.10.002
Journal volume & issue
Vol. 31, no. 3
pp. 170 – 171

Abstract

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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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