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Case Reports
. 2025 May 28;30(12):103419.
doi: 10.1016/j.jaccas.2025.103419. Epub 2025 Apr 24.

The Arrhythmic Mitral Valve Prolapse Syndrome: A New Insight for Understanding the Arrhythmogenic Substrate?

Rahul Ghelani  1 , Ji-Jian Chow  2 , Alejandra Miyazawa  2 , Jessica Artico  2 , Graham Cole  2 , Prapa Kanagaratnam  2 , Nicholas S Peters  2 , Amanda Varnava  2
Affiliations
Case Reports

The Arrhythmic Mitral Valve Prolapse Syndrome: A New Insight for Understanding the Arrhythmogenic Substrate?

Rahul Ghelani et al. JACC Case Rep. .

Abstract

Background: Mitral valve prolapse (MVP) is the most common valvular heart disease and historically was considered benign in the absence of severe mitral regurgitation and normal left ventricular function. However, an emerging subset associated with sudden cardiac death that does not follow traditional risk stratification has been established termed the arrhythmic mitral valve prolapse (A-MVP) syndrome. This cohort provide a clinical challenge on identifying those at risk of lethal arrhythmias who may benefit from a primary prevention implantable cardiac defibrillator.

Case summary: We present 7 cases in which patients have survived cardiac arrest due to A-MVP, with the aim of further describing phenotypic characteristics that define this syndrome.

Discussion: We observed that after arrest, a high proportion of patients developed rapidly worsening mitral regurgitation. This observed link may provide insight into the underlying substrate of arrhythmogenesis.

Keywords: cardiac magnetic resonance; cardiac risk; echocardiography; electrophysiology; mitral valve; phenotype; primary prevention; ventricular fibrillation; ventricular tachycardia.

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Conflict of interest statement

Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Arrhythmic Mitral Valve Prolapse Phenotype Summary of the observed phenotype in arrhythmic mitral valve prolapse syndrome. CMR = cardiac magnetic resonance; FH = family history; LVEF = left ventricular ejection fraction; LGE = late gadolinium enhancement; NSVT = nonsustained ventricular tachycardia; SCD = sudden cardiac death; TWI = T-wave inversion; VEs = ventricular ectopies.
See this image and copyright information in PMC

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