Arrhythmogenic Right Ventricular Cardiomyopathy/ Dysplasia

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an under diagnosed idiopathic progressive cardiomyopathy, associated with mutations in genes coding for desmosomal proteins, with well-studied hereditary mechanisms in some populations. The Task Force 2010 defined diagnostic criteria for ARVC/D: structural (by echocardiography and cardiac magnetic resonance imaging); histopathological (if biopsy is required); electrical (by electrocardiography, exercise testing and Holter monitoring) and genetic/ familial. When those criteria are met, the associated sudden death risk should be then tackled by EPS, leading to the eventual insertion of an Implantable Cardioverter Defibrillator. Genetic association studies should be offered to ARVC/D patient's offspring.