17
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found

      Ventricular fibrillation refractory to automatic internal cardiac defibrillator in Fabry's disease. Review of cardiovascular manifestations.

      1 , , ,
      Cardiology
      S. Karger AG

      Read this article at

      ScienceOpenPublisherPubMed
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Fabry's disease is a disorder of glycosphingolipid metabolism leading to alpha-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.

          Related collections

          Author and article information

          Journal
          Cardiology
          Cardiology
          S. Karger AG
          0008-6312
          0008-6312
          2000
          : 94
          : 3
          Affiliations
          [1 ] Department of Cardiology and Pathology, Tripler Army Medical Center, Honolulu, HI 96859-5000, USA. robert_e.eckhart@tamc.chcs.amedd.army.mil
          Article
          47318
          10.1159/000047318
          11279328
          eb1ef3e3-ebf0-430b-8d8e-8c4def8da6fd
          History

          Comments

          Comment on this article