Arrhythmogenic cardiomyopathy (ACM) is a form of inheritable cardiomyopathy characterized by ventricular scarring and/or fibro-fatty replacement. ACM is associated with a significant risk of sudden cardiac death due to ventricular arrhythmias, especially in young patients and endurance athletes. Aim of this review is to summarize the changing in understanding and framing of this multifaceted disease, in particular highlighting the current transition from a phenotype-first to a genotype-first framework for diagnosis and management.
Keywords: Arrhythmogenic cardiomyopathy; Arrhythmogenic right ventricular cardiomyopathy; Desmosomal; Sudden cardiac death.
© The Author(s) 2026. Published by Oxford University Press on behalf of the European Society of Cardiology.