• Kevin M Harris, Shannon Mackey-Bojack, Giselle Fisher, Darlington Nwaudo, and Barry J Maron.
• Minneapolis Heart Institute Foundation at Abbott-Northwestern Hospital, Minn. Electronic address: kevin.harris@allina.com.
• Am. J. Med. 2024 Aug 13.

BackgroundSudden deaths (SDs) 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.MethodsForty-three MVP-related SDs were identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.ResultsEvents occurred in both sexes, 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.ConclusionsArrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people, including competitive athletes, disproportionally affecting females and 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.Copyright © 2024 Elsevier Inc. All rights reserved.

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