Literature DB >> 26336553

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

Takashi Kanda1, Masaharu Masuda1, Akihiro Sunaga1, Masashi Fujita1, Osamu Iida1, Shin Okamoto1, Takayuki Ishihara1, Kiyonori Nanto1, Tatsuya Shiraki1, Fusako Sera1, Masaaki Uematsu1.   

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.

Entities:  

Keywords:  Fabry disease; High defibrillation thresholds; Implantable cardioverter-defibrillator; Ventricular fibrillation

Year:  2014        PMID: 26336553      PMCID: PMC4550129          DOI: 10.1016/j.joa.2014.10.002

Source DB:  PubMed          Journal:  J Arrhythm        ISSN: 1880-4276


  4 in total

Review 1.  How to manage the patient with a high defibrillation threshold.

Authors:  Sumeet K Mainigi; David J Callans
Journal:  Heart Rhythm       Date:  2006-04       Impact factor: 6.343

2.  QRS prolongation is associated with high defibrillation thresholds during cardioverter-defibrillator implantations in patients with hypertrophic cardiomyopathy.

Authors:  Takayuki Nagai; Takashi Kurita; Kazuhiro Satomi; Takashi Noda; Hideo Okamura; Wataru Shimizu; Kazuhiro Suyama; Naohiko Aihara; Junjiro Kobayashi; Shiro Kamakura
Journal:  Circ J       Date:  2009-04-10       Impact factor: 2.993

3.  Increased defibrillation threshold with right-sided active pectoral can.

Authors:  P R Roberts; S Allen; T Betts; J M Morgan; J F Urban; T Whitman; D E Euler; M J Kallok
Journal:  J Interv Card Electrophysiol       Date:  2000-04       Impact factor: 1.900

4.  Arrhythmias in Fabry cardiomyopathy.

Authors:  Deepak Acharya; Peter Robertson; G Neal Kay; Leslie Jackson; David G Warnock; Vance J Plumb; José A Tallaj
Journal:  Clin Cardiol       Date:  2012-08-09       Impact factor: 2.882

  4 in total

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