Arrhythmogenic Mitral Valve Prolapse: Looking Beyond Mitral Regurgitation

Mitral valve prolapse (MVP) is usually evaluated through the severity of mitral regurgitation (MR), yet some patients may exhibit an arrhythmogenic phenotype associated with life-threatening ventricular arrhythmias.

A 70-year-old asymptomatic woman with Barlow disease was referred for MR reassessment. Echocardiography showed bileaflet prolapse, moderate to severe MR, mitral annular disjunction, and left-sided chamber remodeling. Ambulatory electrocardiographic monitoring revealed frequent polymorphic premature ventricular complexes. Before work-up completion, she experienced an out-of-hospital ventricular fibrillation with cardiac arrest. Cardiac magnetic resonance demonstrated inferolateral myocardial fibrosis. She underwent implantable cardioverter-defibrillator placement, followed by mitral valve repair and surgical ablation.

This case highlights the need to identify arrhythmogenic MVP early, using an integrated clinical, electrocardiographic, echocardiographic, and cardiac magnetic resonance approaches for individualized risk stratification.

In patients with MVP, arrhythmic risk stratification should go beyond MR severity, integrating red flags with clinical assessment, serial multimodality imaging, and a multidisciplinary team involving imaging, electrophysiology, and surgical expertise.