Arrhythmic mitral valve prolapse: In which patients should primary prevention of sudden cardiac death be considered and how should it be implemented?

Mitral valve prolapse (MVP) affects 2-3 % of the population and is generally benign. However, a subgroup presents severe complications such as ventricular arrhythmias and sudden cardiac death (SCD). Arrhythmogenic mitral valve prolapse (AMVP) combines MVP with frequent or complex ventricular arrhythmias in the absence of other causes. Although SCD in AMVP patients is rare (0.2-0.4 %, annual), it exceeds the general population rate and is associated with specific high arrhythmic risk features, such as mitral annular disjunction (MAD), bileaflet prolapse, Pickelhaube sign, abnormal T waves on the electrocardiogram, and rapid non-sustained ventricular tachycardia. Primary prevention is crucial but challenged by the lack of standardized guidelines. AMVP requires a multidisciplinary and cost-effective evaluation to stratify risk and prevent SCD in a predominantly young and healthy population. The objective of this review is to describe the variables with the highest arrhythmogenic risk in patients with MVP and the possible primary prevention strategies for sudden cardiac death.