Current cardiology reports
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The Brugada syndrome, first described as a new clinical entity in 1992, is widely recognized today as a form of inherited sudden cardiac arrest. The past 16 years witnessed a progressive increase in the number of reported cases and a dramatic proliferation of articles serving to define the clinical, genetic, cellular, ionic, and molecular aspects of the disease. This article provides a brief overview of recent advances in our understanding of the clinical presentation and molecular and cellular mechanisms and an update of existing controversies.
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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). The diagnosis is based on the International Task Force criteria. Cardiologists may not be aware of these diagnostic criteria for ARVC/D and may place too much importance on the results of MRI imaging of the right ventricle. ⋯ We also recommend treatment with beta blockers. Patients with ARVC/D are encouraged to avoid competitive athletics. Recent advances in the understanding of the genetic basis of ARVC/D have revealed that ARVC/D is a disease of desmosomal dysfunction.