Mitral valve prolapse syndrome as cause of sudden death in young adults

Abstract

Mitral valve prolapse syndrome (MVPS) is a relatively common disorder of the mitral valve and most cases take a benign clinical course. Only a subset of patients develop severe clinical symptoms such as arrhythmia, insufficiency of the mitral valve or infective endocarditis. As a consequence, sudden death might occur in these patients, thought to be caused by an arrhythmogenic event. By presenting six cases of sudden unexpected death in young female adults, we point at clinical and pathological data from the literature, which are of interest from the viewpoint of legal medicine. The incidence of MVPS in autopsy series has been reported to be about 4-5%, while clinical data hint at an incidence of about 2.5%. The presented cases suggest that even clinically benign cases of MVPS in young adults might result in sudden unexpected death. Such cases are not included in hospital based studies on the topic. This might lead to an underestimation of the fatal risk associated with the disease, even if sudden death might be a rare event in MVPS.