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Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterized by fibro-fatty myocardial replacement, clinically associated with malignant ventricular arrhythmias and sudden cardiac death. Originally described a disease with a prevalent right ventricular (RV) involvement, subsequently two other phenotypes have been recognized, such as the left dominant and the biventricular phenotypes, for which a recent International Expert consensus document provided upgrade diagnostic criteria (the 2020 "Padua Criteria"). In this novel workup for the diagnosis of the entire spectrum of phenotypic variants of ACM, including left ventricular (LV) variants, cardiac magnetic resonance (CMR) has emerged as the cardiac imaging technique of choice, due to its capability of detailed morpho-functional and tissue characterization evaluation of both RV and LV. In this review, the key role of CMR in the diagnosis of ACM is outlined, including the supplemental value for the characterization of the disease variants. An ACM-specific CMR study protocol, as well as strengths and weaknesses of each imaging technique, is also provided. Key Points: • Arrhythmogenic cardiomyopathy includes three different phenotypes: dominant right, biventricular, and dominant left. • In 2020, diagnostic criteria have been updated and cardiac magnetic resonance has emerged as the cardiac imaging technique of choice. • This aim of this review is to provide an update of the current state of art regarding the use of CMR in ACM, with a particular focus on novel diagnostic criteria, CMR protocols, and prognostic significance of CMR findings in ACM. [ABSTRACT FROM AUTHOR]

Arrhythmic mitral valve prolapse (MVP) is an increasingly recognized clinical entity, characterized by the association of myxomatous mitral valve, ventricular arrhythmias (VAs) and sudden cardiac death (SCD). Prevalence of MVP is reported ranging between 2% and 5% of the general population, and risk of SCD is estimated approximately 0.3% per year. Diagnosis of MVP and the occurrence of fatal events involve generally adults aged 30 to 50 years, whereas in younger and even pediatric individuals has rarely been described. Herein, we report two clinical cases of malignant MVP in young patients, with the aim to point out the clinical features and the challenge of clinical management and risk stratification. [ABSTRACT FROM AUTHOR]