Arrhythmic Mitral Valve Prolapse: JACC Review Topic of the Week

There is an increasing awareness of the association between mitral valve prolapse and sudden cardiac death. There are several clinical risk factors associated with an increased risk of mitral valve prolapse–related sudden cardiac death, most of which can be evaluated with noninvasive diagnostic modalities. For example, characteristic changes on the electrocardiogram (T-wave inversions in the inferior leads), complex ventricular ectopy, a spiked configuration of the lateral annular velocities by echocardiography, and evidence of myocardial fibrosis by cardiac magnetic resonance imaging have all been implicated as markers of risk. Herein, the authors review the reported incidence of sudden death to mitral valve prolapse, the clinical profile of at-risk patients, and the basic components necessary to initiate and perpetuate ventricular arrhythmias (substrate and trigger) as well as potential interventions to consider for those at highest risk.