Contemporary diagnostic approach to arrhythmogenic cardiomyopathy: The three-step work-up.

ABSTRACT

Arrhythmogenic Cardiomyopathy (ACM) is a cardiac disorder characterized by non-ischemic myocardial scarring, which may lead to ventricular electrical instability and systolic dysfunction. Diagnosing ACM is challenging as there is no single gold-standard test and a combination of criteria is required. The first diagnostic criteria were established in 1994 and revised in 2010, focusing primarily on right ventricular involvement. However, in 2019, an international expert report identified limitations of previous diagnostic scoring and developed the 2020 Padua criteria with also included criteria for diagnosis of left ventricular variants and introduced cardiac magnetic resonance tissue characterization findings for detection of left ventricular myocardial scar. These criteria were further refined and published in 2023 as the European Task Force criteria, gaining international recognition. This review provides an overview of the 20 years of progresses on the disease diagnostic from the original 1994 criteria to the most recent 2023 European criteria, highlighting the evolution into our understanding of the pathobiology and morpho-functional features of the disease.