Arrhythmogenic Right Ventricular Cardiomyopathy.

Arrhythmogenic disorders of genetic origin include structural cardiomyopathies and inherited arrhythmic syndromes(1). Arrhythmogenic right ventricular cardiomyopathy (ARVC) is listed among the former, since it is characterized by structural and functional abnormalities of the right ventricle (RV), ranging from regional wall motion abnormalities and ventricular aneurysms, to global ventricular dilation and dysfunction, with or without left ventricular (LV) involvement(2-4). The clinical picture is usually dominated by ventricular arrhythmias, even at risk of sudden cardiac death (SCD) in young people during effort(5-7), thus justifying the adjective "arrhythmogenic". However, it has been recently emphasized that in the early stages (concealed phase) the disease can resemble ion channel diseases, such as long QT and Brugada syndromes, since it can carry the risk of ventricular arrhythmias before the onset of overt structural abnormalities. Thus, early diagnosis and risk stratification pose major clinical challenges along the natural history of ARVC