Implantable defibrillators in long QT syndrome, Brugada syndrome, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy

Cardiol Clin. 2014 May;32(2):305-18. doi: 10.1016/j.ccl.2013.11.003. Epub 2014 Jan 23.

Abstract

Sudden death is often the first manifestation in inherited cardiac arrhythmia syndromes. Patients with long QT syndrome who have an episode of syncope while on beta-blockade should be offered an implantable cardioverter-defibrillator (ICD). In Brugada syndrome and hypertrophic cardiomyopathy, ICDs are often the most effective treatment of primary and secondary prevention of cardiac arrest. Risk stratification is crucial in identifying those at greatest risk to provide lifesaving therapy with an ICD while avoiding complications in those unlikely to receive benefit.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Brugada syndrome; Channelopathy; Genetic syndrome; Hypertrophic cardiomyopathy; Long QT syndrome; Sudden death.

Publication types

  • Review

MeSH terms

  • Adrenergic beta-Antagonists / therapeutic use
  • Arrhythmogenic Right Ventricular Dysplasia / diagnosis
  • Arrhythmogenic Right Ventricular Dysplasia / therapy*
  • Brugada Syndrome / diagnosis
  • Brugada Syndrome / therapy*
  • Cardiomyopathy, Hypertrophic / diagnosis
  • Cardiomyopathy, Hypertrophic / therapy*
  • Death, Sudden, Cardiac / prevention & control
  • Defibrillators, Implantable
  • Humans
  • Long QT Syndrome / diagnosis
  • Long QT Syndrome / therapy*
  • Prosthesis Design
  • Prosthesis Failure
  • Risk Assessment / methods

Substances

  • Adrenergic beta-Antagonists