Arrhythmogenic Mitral Valve Prolapse and Mitral Annular Disjunction: From Observation to Intervention

Arrhythmogenic Mitral Valve Prolapse and Annular Disjunction

Abstract

Ventricular arrhythmias and sudden cardiac death remain rare manifestations of mitral valve prolapse (MVP). Often referred to as arrhythmogenic MVP, this subset of a common valvular pathology has become increasingly recognized and is associated with key characteristics across a variety of imaging modalities. With a better understanding of the relevant anatomical considerations, risk stratification for arrhythmia development is essential to identifying at-risk patients and providing targeted therapies. Patient evaluation should incorporate a thorough review of imaging, including echocardiography and, when necessary, cardiac magnetic resonance imaging, to ensure accurate diagnosis. We present a case series depicting the spectrum of arrhythmogenic MVP severity and methods of risk stratification aimed at identifying characteristics linking valvular pathology to the development of lethal arrhythmias. Based on limited data and our institutional experience, we propose an algorithm for clinical decision-making and management of these patients.