%0 Journal Article
%T Arrhythmogenic Mitral Valve Prolapse Revisited: A Not Uncommon Cause of Youthful Sudden Death in Athletes and Women.
%A Harris KM
%A Mackey-Bojack S
%A Fisher G
%A Nwaudo D
%A Maron BJ
%J Am J Med
%V 0
%N 0
%D 2024 Aug 13
%M 39147083
%F 5.928
%R 10.1016/j.amjmed.2024.08.002
%X <B>BACKGROUND: </B>Sudden deaths (SD) in young people including competitive athletes, albeit uncommon, are usually attributable to genetic, congenital or acquired cardiovascular conditions. However, it is under-appreciated that mitral valve prolapse (MVP), a relatively common valvular heart disease, is associated with SD in this youthful population.<BR><B>METHODS: </B>Forty-three MVP-related SDs are identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.<BR><B>RESULTS: </B>Events occurred in both genders, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist resulting in confirmed MVP diagnosis in 11.. Pathologic findings characteristic of MVP included: bileaflet myxomatous involvement in all cases; and areas of interstitial or replacement myocardial fibrosis in 79%, most evident in posterolateral left ventricular wall.<BR><B>CONCLUSIONS: </B>Arrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people including competitive athletes, disproportionally affecting females and requiring requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.