Atrial tachyarrhythmias in arrhythmogenic cardiomyopathy

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by replacement of ventricular myocardial by fibrofatty scar tissue that underlies morpho-functional ventricular abnormalities and life-threatening ventricular arrhythmias potentially responsible for sudden cardiac death, mostly in young people and athletes.1,2 Although the original disease phenotype was characterized by predominant right ventricular (RV) involvement (arrhythmogenic right ventricular cardiomyopathy [ARVC]), the increasing identification of biventricular and left-dominant forms has led to use the term "arrhythmogenic cardiomyopathy," which better defines the broader spectrum of the disease phenotypic expression with fibrofatty myocardial scar involvement of both ventricles.