Two Cases of Arrhythmic Mitral Valve Prolapse: Evil Has Many Faces

Mitral valve prolapse (MVP) is one of the most common valvular heart diseases. Although MVP is generally considered benign, it can be associated with important complications, including sudden cardiac death (SCD), owing to ventricular arrhythmias (VAs). Several clinical, electrocardiographic, and imaging findings have been associated with MVP-related SCD, including female sex, T-wave inversions in the inferior leads, complex ventricular ectopy, leaflet redundancy (classic MVP), mitral annular disjunction, pickelhaube sign (a spiked configuration of the lateral annular velocities), and evidence of myocardial fibrosis in cardiac magnetic resonance (CMR) imaging. However, neither of these markers, nor any specific combination of them, have proved to be a consistent predictor of malignant VAs and SCD. In this context, we present 2 interesting cases of arrhythmic MVP, highlighting the broad clinical spectrum of this condition, the potential underlying arrhythmogenic mechanisms, and the merit of identifying patients at high arrhythmic risk.