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The association between the mitral valve prolapse and the sudden Cardiac Death remains controversial, the high prevalence of this valvulopathy contrasting with the low incidence of sudden death in this population. We report the case of a 54-year-old woman admitted for a sudden cardiac death, revealing a bi-prolapse with low-grade leakage, leading to the implantation of a subcutaneous automatic defibrillator. Combined echocardiography and cardiac MRI can identify the mitral annular disjunction, the rolling motion of the posterior face of the mitral annulus towards the myocardium, and the myocardial fibrosis of the inferolateral wall induced by streching forces of the sub valvular apparatus, that may lead to ventricular arrhythmias. More than the conventional clinical parameters (young woman, ventricular premature beats with a right bundle branch block morphology, mitral bi-prolapse), mitral annular disjunction and myocardial fibrosis are to be considered as powerful markers of the rhythmic risk of mitral prolapse and must be systematically sought and integrated into the prognostic evaluation of these patients. In the absence of randomised trials, therapeutic management is difficult especially in primary prevention, and needs Heart Team advice.

The association between the mitral valve prolapse and the sudden Cardiac Death remains controversial, the high prevalence of this valvulopathy contrasting with the low incidence of sudden death in this population. We report the case of a 54-year-old woman admitted for a sudden cardiac death, revealing a bi-prolapse with low-grade leakage, leading to the implantation of a subcutaneous automatic defibrillator. Combined echocardiography and cardiac MRI can identify the mitral annular disjunction, the rolling motion of the posterior face of the mitral annulus towards the myocardium, and the myocardial fibrosis of the inferolateral wall induced by streching forces of the sub valvular apparatus, that may lead to ventricular arrhythmias. More than the conventional clinical parameters (young woman, ventricular premature beats with a right bundle branch block morphology, mitral bi-prolapse), mitral annular disjunction and myocardial fibrosis are to be considered as powerful markers of the rhythmic risk of mitral prolapse and must be systematically sought and integrated into the prognostic evaluation of these patients. In the absence of randomised trials, therapeutic management is difficult especially in primary prevention, and needs Heart Team advice.