Key result
This review describes the current understanding of the molecular genetics of inherited arrhythmic diseases, specifically long QT syndrome and Brugada syndrome.
This review summarizes the molecular genetics underlying long QT syndrome and Brugada syndrome, which are important causes of sudden cardiac death in structurally normal hearts.
May support new targets in arrhythmogenic death; leaves open prospective validation before practice change.
Sudden cardiac death is a significant problem in the United States, with an incidence reported to be greater than 300,000 persons per year [1]. Interest in identifying the underlying cause of the death has been focused on cases of unexpected arrhythmogenic death, which is estimated to represent 5% of all sudden deaths. In cases in which no structural heart disease can be identified, the long QT syndrome and ventricular pre-excitation are most commonly considered as likely causes. Brugada syndrome (also known by some investigators as idiopathic ventricular fibrillation [IVF]) [2], is a disease associated with an electrocardiographic abnormality of right bundle branch block (RBBB) with ST-elevation in the right precordial leads (V1-V3). Recently it has been added to the list of possible causes of sudden death in otherwise healthy, young individuals. The purpose of this paper is to describe the current understanding of the molecular genetics of inherited diseases in which arrhythmias are prominent features, specifically long QT syndrome and Brugada syndrome.
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Towbin et al. (1999) conducted a review in Long QT syndrome and Brugada syndrome. Molecular genetics was evaluated. This review describes the current understanding of the molecular genetics of inherited arrhythmic diseases, specifically long QT syndrome and Brugada syndrome.
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