Arrhythmogenic Mitral Valve Prolapse Revisited: A Not Uncommon Cause of Youthful Sudden Death in Athletes and Women - 18/12/24

Doi : 10.1016/j.amjmed.2024.08.002

Kevin M. Harris, MD ^a,^⁎ , Shannon Mackey-Bojack, MD ^b, Giselle Fisher, BS ^a, Darlington Nwaudo, MD ^a, Barry J. Maron, MD ^c
^a Minneapolis Heart Institute Foundation at Abbott-Northwestern Hospital, Minn
^b Jesse E. Edwards Registry of Cardiovascular Disease, St. Paul, Minn
^c Lahey Hospital and Medical Center, Burlington, Mass

^⁎Requests for reprints should be addressed to Kevin M. Harris, MD, Minneapolis Heart Institute, 920 E. 28th Street, Suite 300, Minneapolis, MN, 55407.Minneapolis Heart Institute920 E. 28th Street, Suite 300MinneapolisMN55407

Abstract

Background

Sudden 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.

Methods

Forty-three MVP-related SDs were identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.

Results

Events 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.

Conclusions

Arrhythmogenic 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.

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Keywords : Athletes, Mitral valve prolapse, Sudden death

Plan

	Funding: None.
	Conflict of Interest: None.
	Authorship: All authors had access to the data and a role in writing this manuscript. KMH: Writing – review & editing, Writing – original draft, Supervision, Methodology, Investigation, Formal analysis, Data curation, Conceptualization; SMB: Writing – review & editing, Supervision, Resources, Methodology, Investigation, Formal analysis, Data curation, Conceptualization; GF: Methodology, Data curation, Conceptualization; DN: Writing – review & editing, Investigation, Formal analysis, Conceptualization; BJM: Writing – review & editing, Writing – original draft, Validation, Resources, Investigation, Formal analysis, Data curation, Conceptualization.