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Sudden death and ion channel disease: pathophysiology and implications for management
  1. Rachel Bastiaenen,
  2. Elijah R Behr
  1. Department of Cardiovascular Sciences, St George's University of London, London, UK
  1. Correspondence to Elijah R Behr, St George's University of London, Division of Cardiovascular Sciences, Cranmer Terrace, London SW17 0RE, UK; ebehr{at}


The underlying aetiology of sudden arrhythmic death syndrome is predominantly inherited cardiac disease, and ‘channelopathies’ (cardiac ion channel disease) are the most common detectable cause of death. This heterogeneous group includes Brugada syndrome, long QT syndrome and catecholaminergic polymorphic ventricular tachycardia. Common features include variable penetrance, sudden death due to ventricular arrhythmias, and the absence of structural heart disease. The understanding of cardiac ion channel disease has been revolutionised by genetics. At present, genotype contributes to risk stratification in Brugada syndrome, long QT syndrome and catecholaminergic polymorphic ventricular tachycardia, and the future promises management tailored to the genetic diagnosis.

  • Sudden adult death syndrome
  • genetics
  • risk stratification

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  • Funding Funds for research received from Biotronik and Boston Scientific.

  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.