Dilated cardiomyopathy (DCM) is a myocardial disorder defined by ventricular chamber enlargement and systolic dysfunction. DCM can result in progressive heart failure, arrhythmias, thromboembolism, and premature death, and contributes significantly to health care costs. In many cases, DCM results from acquired factors that affect cardiomyocyte function or survival. Inherited genetic variants are also now recognized to have an important role in the etiology of DCM. Despite substantial progress over the past decade, our understanding of familial DCM remains incomplete. Current concepts of the molecular pathogenesis, clinical presentation, natural history, and management of familial DCM are outlined in this review.
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