Arrhythmogenic right ventricular cardiomyopathy is a relatively well-defined clinical entity. This disease is characterized with right ventricular involvement and is an important cause of sudden cardiac death in young patients. However, arrhythmogenic cardiomyopathy with left-dominant involvement has recently been better described in the literature. This new presentation may be confused with other diseases such as idiopathic dilated cardiomyopathy. This review outlines left-dominant arrhythmogenic cardiomyopathy in the light of the most recent information.
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