Arrhythmogenic right ventricular cardiomyopathy/dysplasia

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited cardiomyopathy characterized clinically by ventricular arrhythmias, sudden death, and structural abnormalities of the right ventricle. Although structural involvement of the right ventricle predominates, a left dominant form (LDAC) has been described. Structural involvement of both ventricles is common in advanced disease. Due to significant heterogeneity in its manifestation, the diagnosis of ARVC/D is challenging and requires a multifaceted approach to patient evaluation. The recently published Revised Task Force Criteria provide clinicians with a validated model to improve the diagnosis of ARVC/D. The management of ARVC/D is primarily aimed at reducing the burden of symptomatic arrhythmias and decreasing the incidence of sudden cardiac death. Automatic implantable cardioverter-defibrillators (AICDs) significantly reduce mortality in patients with ARVC/D. However, accurate risk assessment is needed to avoid exposing low-risk patients to the long-term complications of AICDs. Strenuous exertion increases the rate of disease progression and increases the risk of sudden death.