Home > Electrophysiology, Journal Club > Journal Club 19 August 2015

Journal Club 19 August 2015

Paper

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

Presenter

SH

Summary

Background

Mitral valve prolapse (MVP) may present with ventricular arrhythmias and sudden cardiac death (SCD) even in the absence of hemodynamic impairment. The structural basis of ventricular electric instability remains elusive.

Methods and Results

The cardiac pathology registry of 650 young adults (≤40 years of age) with SCD was reviewed, and
cases with MVP as the only cause of SCD were re-examined. Forty-three patients with MVP (26 females; age range,19–40 years; median, 32 years) were identified (7% of all SCD, 13% of women). Among 12 cases with available ECG, 10 (83%) had inverted T waves on inferior leads, and all had right bundle-branch block ventricular arrhythmias. A bileaflet involvement was found in 70%. Left ventricular fibrosis was detected at histology at the level of papillary muscles in
all patients, and inferobasal wall in 88%. Living patients with MVP with (n=30) and without (control subjects; n=14) complex ventricular arrhythmias underwent a study protocol including contrast-enhanced cardiac magnetic resonance. Patients with either right bundle-branch block type or polymorphic complex ventricular arrhythmias (22 females; age range, 28–43 years; median, 41 years), showed a bileaflet involvement in 70% of cases. Left ventricular late enhancement
was identified by contrast-enhanced cardiac magnetic resonance in 93% of patients versus 14% of control subjects (P<0.001), with a regional distribution overlapping the histopathology findings in SCD cases.

Conclusions

MVP is an underestimated cause of arrhythmic SCD, mostly in young adult women. Fibrosis of the
papillary muscles and inferobasal left ventricular wall, suggesting a myocardial stretch by the prolapsing leaflet, is the structural hallmark and correlates with ventricular arrhythmias origin. Contrast-enhanced cardiac magnetic resonance may help to identify in vivo this concealed substrate for risk stratification.

Supplementary Material

The editorial is available here.

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