Genetics of brugada, long QT, and arrhythmogenic right ventricular dysplasia syndromes.
This article outlines the up-to-date understanding of the molecular basis of primary ventricular arrhythmias. Two disorders have recently been well described at the molecular level, the long QT syndromes and Brugada syndrome, and this article reviews the current scientific knowledge of each disease. A third disorder, arrhythmogenic right ventricular dysplasia, which is on the cusp of understanding, will also be described.
Department of Pediatrics (Cardiology), Texas Children's Hospital and Baylor College of Medicine, Houston 77030, USA. jtowbin@bcm.tmc.edu
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Towbin
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Vatta
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Li
D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D002037:Bundle-Branch Block / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004562:Electrocardiography; D006801:Humans; D007473:Ion Channels / Q000235:genetics; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D009154:Mutation / Q000235:genetics; D013577:Syndrome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology
D007473:Ion Channels