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      Ventricular Fibrillation Refractory to Automatic Internal Cardiac Defibrillator in Fabry’s Disease

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          Abstract

          Fabry’s disease is a disorder of glycosphingolipid metabolism leading to α-galactosidase deficiency with systemic sequelae. Clinical cardiac manifestations include dysrhythmias, structural abnormalities apparent on echocardiography, and histologic changes secondary to glycosphingolipid deposition. The introduction of automated internal cardiac defibrillators (AICD) has been shown to decrease the incidence of circulatory collapse in individuals with known terminal arrhythmias. We present a patient with Fabry’s disease, who underwent coronary angiography without finding of obstructive disease. He returned after aborted sudden cardiac death necessitating the placement of an AICD. He again presented after an episode of ventricular fibrillation refractory to internal defibrillation necessitating advanced life support, and subsequently expired. We review the electrocardiographic, cardiovascular structural, and histologic manifestations of Fabry’s disease.

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          Author and article information

          Journal
          CRD
          Cardiology
          10.1159/issn.0008-6312
          Cardiology
          S. Karger AG
          0008-6312
          1421-9751
          2000
          February 2001
          02 March 2001
          : 94
          : 3
          : 208-212
          Affiliations
          Department of Cardiology and Pathology, Tripler Army Medical Center, Honolulu, Hawaii, USA
          Article
          47318 Cardiology 2000;94:208–212
          10.1159/000047318
          11279328
          © 2001 S. Karger AG, Basel

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          Page count
          Figures: 3, References: 18, Pages: 5
          Categories
          Case Report

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