Mitral Valve Prolapse and Mitral Annular Disjunction Arrhythmic Syndromes: Diagnosis, Risk Stratification and Management

Panagioula Niarchou, Efstathia Prappa, Ioannis Liatakis, Konstantinos Vlachos, Anastasios Chatziantoniou, Eva Nyktari, Gary Tse, Michael Efremidis, Konstantinos P. Letsas

Research output: Contribution to journalReview articlepeer-review

4 Citations (Scopus)

Abstract

Although mitral valve prolapse (MVP) is usually considered a benign clinical condition, it has been linked with ventricular arrhythmias and sudden cardiac death in patients with a certain “arrhythmic” phenotype, raising awareness and mandating a specific risk stratification protocol. Mitral annular disjunction (MAD) is considered a “red flag” in malignant MVP syndrome along with bileaflet myxomatous prolapse, female gender, negative or biphasic T waves in the inferior leads, fibrosis in the papillary muscles or inferobasal wall detected by cardiac magnetic resonance imaging and complex arrhythmias of right bundle branch morphology. MAD seems to play a critical role in the chain of morphofunctional abnormalities which lead to increased mechanical stretch and subsequent fibrosis mainly in the papillary muscles, forming the vulnerable anatomic substrate prone to arrhythmogenesis, and associated with long-term severe ventricular arrhythmias. Arrhythmogenesis in MVP/MAD patients is not fully understood but a combination between a substrate and a trigger has been established with premature ventricular contraction triggered ventricular fibrillation being the main mechanism of sudden cardiac death (SCD). Certain characteristics mostly recognized by non-invasive imaging modalities serve as risk factors and can be used to diagnose and identify high risk patients with MAD, while treatment options include catheter ablation, device therapy and surgical intervention. This review focuses on the clinical presentation, the arrhythmogenic substrate, and the incidence of ventricular arrhythmias and SCD in MAD population. The current risk stratification tools in MAD arrhythmogenic entity are discussed.

Original languageEnglish
Article number295
JournalReviews in Cardiovascular Medicine
Volume23
Issue number9
DOIs
Publication statusPublished - Sept 2022
Externally publishedYes

Keywords

  • mitral annular disjunction
  • mitral valve prolapse
  • sudden cardiac death
  • ventricular arrhythmias

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