The management of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) has evolved considerably over the last several years. Because many patients with ARVC/D may be at risk for sudden cardiac death (SCD) and clinical series have reported favorable outcomes with this therapy, the implantable cardioverter-defibrillator (ICD) has assumed a larger role in therapy. On the basis of collective observations from several series of patients, implantation of an ICD in a patient with ARVC/D has a major impact on decreasing the risk of SCD. This chapter examines the available clinical information regarding ICD therapy in patients with ARVC/D.
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