与二尖瓣脱垂（MVP）和导致心源性猝死（SCD）相关的室性心律失常（称为“恶性MVP”）是一种日益被认识的现象，尽管这种现象很少见。 SCD 的发生可能没有明显的二尖瓣反流，这意味着影响二尖瓣装置和左心室的机械紊乱之间存在相互作用。这些心律失常的风险分层是一个重要的临床和公共卫生问题，以提供精确和有针对性的管理。评估需要患者和家族史、体格检查以及电生理学和影像学方法。我们对致心律失常 MVP 进行了综述，探讨了其流行病学、人口统计学、临床表现、将 MVP 与 SCD 联系起来的机制、疾病严重程度的标志物、检测方式和管理，并讨论了风险分层的重要性。即使最近认识有所提高，如何最好地权衡临床、影像和电生理数据的预后重要性以确定明确的高风险心律失常特征，其中 ICD 应用于 SCD 的一级预防，仍然具有挑战性。版权所有 © 作者(s)，2024 年。由 Radcliffe Group Ltd 出版。

Ventricular arrhythmias associated with mitral valve prolapse (MVP) and the capacity to cause sudden cardiac death (SCD), referred to as 'malignant MVP', are an increasingly recognised, albeit rare, phenomenon. SCD can occur without significant mitral regurgitation, implying an interaction between mechanical derangements affecting the mitral valve apparatus and left ventricle. Risk stratification of these arrhythmias is an important clinical and public health issue to provide precise and targeted management. Evaluation requires patient and family history, physical examination and electrophysiological and imaging-based modalities. We provide a review of arrhythmogenic MVP, exploring its epidemiology, demographics, clinical presentation, mechanisms linking MVP to SCD, markers of disease severity, testing modalities and management, and discuss the importance of risk stratification. Even with recently improved understanding, it remains challenging how best to weight the prognostic importance of clinical, imaging and electrophysiological data to determine a clear high-risk arrhythmogenic profile in which an ICD should be used for the primary prevention of SCD.Copyright © The Author(s), 2024. Published by Radcliffe Group Ltd.