Abstract
Arrhythmic mitral valve prolapse (AMVP) has gained great interest recently given the
increasing recognition of its potential role in unexplained cardiac arrest. While
evidence has accumulated to demonstrate the association between AMVP with sudden cardiac
death (SCD), risk stratification and management remain unclear. Physicians are faced
with the challenges of screening for AMVP among MVP patients and the dilemma of when
and how to intervene to prevent SCD in these patients. In addition, there is little
guidance to help approach MVP patients presenting with an otherwise unexplained cardiac
arrest to know whether MVP was the primary cause of cardiac arrest or just an innocent
bystander. Herein we review the epidemiology and definition of AMVP, the risk and
mechanisms of SCD, and summarize the clinical evidence behind risk markers of SCD
and therapeutic interventions that could potentially prevent it. Lastly, we propose
an algorithm that provides guidance as to how to screen for AMVP and what therapeutic
interventions to use. Also, we propose a diagnostic algorithm for approaching patients
with otherwise unexplained cardiac arrest who are found to have MVP.
Mitral valve prolapse (MVP) is a common condition (1-3%) which is typically asymptomatic.
However, individuals with MVP are at risk of chordal rupture, progressive mitral regurgitation,
endocarditis, ventricular arrhythmias and rarely, sudden cardiac death (SCD). There
is evidence from autopsy series and cohorts of survivors of unexplained cardiac arrest
that MVP is more prevalent in these populations, suggesting that it may have a causal
role in cardiac arrest in susceptible individuals. Potential mechanisms include scar-related
re-entry due to scarring of papillary muscles or impact lesions in the left ventricle
due to the collision between redundant mitral leaflets and the left ventricle.
Recently, risk markers have been identified that help predict the small proportion
of patients with MVP who are at risk of SCD. Patients with MVP who have several of
these risk markers or who have survived an otherwise unexplained cardiac arrest are
said to have Arrhythmogenic Mitral Valve Prolapse (AMVP).
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Article info
Publication history
Accepted:
May 16,
2023
Received in revised form:
April 26,
2023
Received:
March 14,
2023
Publication stage
In Press Journal Pre-ProofFootnotes
Funding
None
Disclosures
None
Identification
Copyright
© 2023 Published by Elsevier Inc. on behalf of the Canadian Cardiovascular Society.
