Arrhythmogenic Mitral Valve Prolapse and Sudden Cardiac Death: An Update and Current Perspectives

Mitral valve prolapse (MVP) affects about 2% to 3% of the general population, mostly women , is the most common cause of primary chronic mitral regurgitation (MR) in western countries. The natural history is heterogeneous , widely deter-mined by the severity of MR. Although most patients remain asymptomatic with a near-normal life expec-tancy, approximately 5% to 10 % progress to severe MR. As largely recognized, left ventricular (LV) dys-function due to chronic volume overload per se identi-fies a subgroup at risk of cardiac death. However, there is rising evidence of a link between MVP and life threating ventricular arrhythmias (VAs)/sudden car-diac death (SCD) in a small subset of middle-aged patients without significant MR, heart failure and remodeled hearts. The present review focuses on the underlying mechanism of electric instability and unex-pected cardiac death in this subset of young patients, from the myocardial scarring of the LV infero-lateral wall due to mechanical stretch exerted by the prolaps-ing leaflets and mitral annular disjunction, to the inflammation's impact on fibrosis pathways along with a constitutional hyperadrenergic state. The heteroge-neity of clinical course reveals a necessity of risk strati-fication, preferably through noninvasive multimodality imaging, that will help to identify , prevent adverse scenarios in young MVP patients. (Curr Probl Cardiol 2023;48:101724.)