Abstract
Almost 40 years after the description of arrhythmogenic cardiomyopathy (ACM), arrhythmic risk stratification remains central to patient management. Antiarrhythmic therapy may involve the use of antiarrhythmic drugs as well as invasive tools such as catheter ablation, with the implantation of an implantable cardioverter defibrillator being of utmost importance. Given the wide phenotypic variability of ACM, the first step in arrhythmic risk stratification requires a thorough assessment of clinical, morphological, and electrical parameters. Moreover, in the last years, genetic testing has become increasingly important, not only for family screening but also in determining prognosis. Finally, data from large series of ACM patients have led to the creation of risk calculators, which are now available online for the medical community. While newly available methods for stratifying arrhythmic risk can be useful, the thoughtful clinical decision-making by clinicians with specific expertise in cardiomyopathies remains of fundamental importance. Additionally, as ACM is a progressive disease, arrhythmic risk stratification should be periodically revised based on newly emerging clinical and instrumental parameters.