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Abstract

Mitral valve prolapse (MVP) is increasingly recognized to have an arrhythmic phenotype associated with ventricular arrhythmias and sudden cardiac death independent of mitral regurgitation severity. Mitral annular disjunction (MAD) is a key structural abnormality implicated in abnormal myocardial mechanics and myocardial fibrosis. We describe a patient with MVP and MAD who developed recurrent non-sustained ventricular tachycardia in association with markedly abnormal basal lateral wall longitudinal strain and progressive myocardial fibrosis on cardiac magnetic resonance imaging. Surgical mitral valve repair performed for MAD resulted in resolution of annular disjunction, improvement in regional myocardial strain, and elimination of ventricular arrhythmias. This case highlights the potential role of mitral valve repair in modifying arrhythmogenic substrate in selected patients with arrhythmic MVP.

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