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Purpose of Review: Left ventricular arrhythmogenic cardiomyopathy (ALVC) is a rare and poorly characterized cardiomyopathy that has recently been reclassified in the group of non-dilated left ventricular cardiomyopathies. This review aims to summarize the background, diagnosis, and sudden cardiac death risk in patients presenting this cardiomyopathy. Recent Findings: Although there is currently a lack of data on this condition, arrhythmogenic left ventricular dysplasia can be considered a specific disease of the left ventricle (LV). We have collected the latest evidence about the management and the risks associated with this cardiomyopathy. Summary: Left ventricular arrhythmogenic cardiomyopathy is still poorly characterized. ALVC is characterized by fibrofatty replacement in the left ventricular myocardium, with variable phenotypic expression. Diagnosis is based on a multiparametric approach, including cardiac magnetic resonance (CMR) and genetic testing, and is important for sudden cardiac death (SCD) risk stratification and management. Recent guidelines have improved the management of left ventricular arrhythmogenic cardiomyopathy. Further studies are necessary to improve knowledge of this cardiomyopathy. [ABSTRACT FROM AUTHOR]

Sudden cardiac death (SCD) is one of the leading causes of cardiovascular death in general population. SCD primary prevention requires the correct selection of patients at increased risk who may benefit from implantable cardioverter-defibrillator (ICD). Despite several noninvasive arrhythmic risk indexes are available, their ability to stratify the SCD risk among asymptomatic patients with cardiac disease at increased arrhythmic risk is debated. The programmed ventricular stimulation (PVS) is an invasive approach historically used for SCD risk stratification in patients with acquired or inherited cardiac disease and is currently included in international guidelines. Aim of this review is to summarize all available data about the role of PVS for the SCD risk stratification in different clinical settings. [ABSTRACT FROM AUTHOR]