The Arrhythmic Mitral Valve Prolapse Syndrome: A New Insight for Understanding the Arrhythmogenic Substrate?

Background: Mitral valve prolapse (MVP) is the most common valvular heart disease and historically was considered benign in the absence of severe mitral regurgitation and normal left ventricular function. However, an emerging subset associated with sudden cardiac death that does not follow traditional risk stratification has been established termed the arrhythmic mitral valve prolapse (A-MVP) syndrome. This cohort provide a clinical challenge on identifying those at risk of lethal arrhythmias who may benefit from a primary prevention implantable cardiac defibrillator.

Case Summary: We present 7 cases in which patients have survived cardiac arrest due to A-MVP, with the aim of further describing phenotypic characteristics that define this syndrome.

Discussion: We observed that after arrest, a high proportion of patients developed rapidly worsening mitral regurgitation. This observed link may provide insight into the underlying substrate of arrhythmogenesis.