Key result
Cardiac ion channel diseases, including Brugada syndrome and long QT syndrome, are the most common detectable causes of sudden arrhythmic death syndrome, with genetics guiding risk stratification.
Cardiac ion channel diseases are a major cause of sudden arrhythmic death syndrome, and genetic testing is increasingly important for risk stratification and tailored management.
Supports genetic testing in SADS evaluation; leaves open prospective validation of risk models.
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.
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Bastiaenen et al. (2011) conducted a review in Sudden arrhythmic death syndrome and cardiac ion channel disease. Cardiac ion channel diseases, including Brugada syndrome and long QT syndrome, are the most common detectable causes of sudden arrhythmic death syndrome, with genetics guiding risk stratification.
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