EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex in collaboration with the ESC Council on valvular heart disease and the European Association of Cardiovascular Imaging endorsed cby the Heart Rhythm

Mitral valve prolapse (MVP) is the most common valvular heart disease,affecting about 2–3% of the general population and is well characterized by echocardiography. While the outcome in MVP is mostly benign in the absence of mitral regurgitation (MR) and its left-ventricular (LV) consequences, a small yet poorly defined subset of individuals remain at higher risk of malignant ventricular arrhythmias (VAs) and sudden cardiac death (SCD). This link between MVP and SCD is reported with annual incidence <1% in unselected individuals with MVP. However, at autopsy, the prevalence of MVP among young patients with sudden arrhythmic death is reported between 4% to up to 7%. Due to the low event-rate and the lack of very large cohorts, assessing the precise incidence of SCD in MVP in general and in specific subsets of patients remains challenging. Mitral annular disjunction (MAD, discussed in detail in section 4) is often observed concomitantly with MVP. MAD results in an abnormal motion of the mitral annulus, termed curling. It is associated with increased risk of arrhythmias and is therefore and integral component of the arrhythmic MVP (AMVP) complex. Over the last decade, a multidisciplinary approach has been employed to identify specific MVP subsets with high arrhythmic risk by careful examination of electrocardiograms (ECG), Doppler Eechocardiography, cardiac magnetic resonance (CMR) imaging, cardiac computed tomography imaging (CT) and confirmed by autopsy findings.