Arrhythmogenic right ventricular cardiomyopathy: Arrhythmogenic right ventricular cardiomyopathy: Clinical assessment and differential diagnosis

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiovascular disorder leading to life-threatening ventricular arrhythmias, progressive biventricular dysfunction, and heart failure. Sudden death can be the unique feature of the disease. Genetic studies indicate that ARVC should be considered a disease of desmosome dysfunction. Diagnosis remains a clinical challenge mainly in its early stages and in patients with minimal imaging structural abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow tract ventricular tachycardia, Brugada syndrome, dilated cardiomyopathy, and myocarditis, due to arrhythmic expressivity and biventricular involvement. Diagnosis is based on major and minor criteria listed in the Revised Task Force Criteria.