title: Arrhythmia-related workup in hereditary myopathies.
abstract: BACKGROUND
  Arrhythmias determine life expectancy in patients with hereditary myopathies.
  
  
  OBJECTIVE
  The aim of this study was to summarize recent advances in the diagnosis and management of arrhythmias in hereditary myopathies.
  
  
  METHODS
  Literature search via PubMed and inclusion of own experiences were performed.
  
  
  RESULTS
  All types of arrhythmias can be found in patients with hereditary myopathies, but some are more prevalent than others. Arrhythmias reported in myopathies include atrial fibrillation, atrial flutter, sick-sinus syndrome, preexcitation syndromes, atrioventricular conduction delay, intraventricular conduction delay, and ventricular tachycardia. Sudden cardiac death is a common finding in certain myopathies, and patients at risk for ventricular arrhythmias and sudden cardiac death should be identified early enough to implant a cardioverter-defibrillator to prevent a fatal outcome. Myopathies associated with a high risk for arrhythmias include laminopathies, Emery-Dreifuss muscular dystrophy, myotonic dystrophy I, mitochondrial myopathies, fatty-acid oxidation defects, and dystrophinopathies. To detect arrhythmias with high risk for sudden cardiac death, patients require close follow-up investigations or an implantable loop recorder. Documentation of severe arrhythmias requires immediate treatment according to established guidelines.
  
  
  CONCLUSIONS
  Patients with certain hereditary myopathies carry an increased risk for developing severe supraventricular or ventricular arrhythmias and for dying of sudden cardiac death. Close follow-up and long-term surveillance of the electrocardiogram may prevent fatal complications of arrhythmias in these patients.
authors.affiliation: Krankenanstalt Rudolfstiftung, Vienna, Austria; Danube University Krems, Krems, Austria. Electronic address: fifigs1@yahoo.de.
authors.forename: Josef
authors.initials: J
authors.lastname: Finsterer
authors.affiliation: Second Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
authors.forename: Claudia
authors.initials: C
authors.lastname: Stöllberger
authors.affiliation: Second Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
authors.forename: Hans
authors.initials: H
authors.lastname: Keller
mesh_terms: D001145:Arrhythmias, Cardiac / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D006329:Heart Conduction System / Q000503:physiopathology; D006801:Humans; D009135:Muscular Diseases / Q000150:complications* / Q000235:genetics* / Q000503:physiopathology; D012307:Risk Factors