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Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death
Cristina Basso1, Martina Perazzolo Marra2, Stefania Rizzo2
1From Departments of Cardiac, Thoracic, and Vascular Sciences (C.B., M.P.M., S.R., M.D.L., A.C., A.C.F., I.R. F.M., K.P., E.B., L.C., B.B., D.C., G.T., S.I.) and Radiology (B.G.), Azienda Ospedaliera-University of Padua Medical School, Padua, Italy. cristina.basso@unipd.it.
Circulation
|July 11, 2015
Summary
Mitral valve prolapse (MVP) is a significant cause of sudden cardiac death (SCD) in young women. Fibrosis in the left ventricle, particularly papillary muscles, is the key structural abnormality linked to ventricular arrhythmias.
Area of Science:
- Cardiology
- Cardiac Electrophysiology
- Pathology
Background:
- Mitral valve prolapse (MVP) can lead to ventricular arrhythmias and sudden cardiac death (SCD), even without significant hemodynamic compromise.
- The underlying structural causes of this electrical instability in MVP are not well understood.
Purpose of the Study:
- To investigate the structural basis of ventricular arrhythmias and SCD in patients with MVP.
- To identify imaging biomarkers for risk stratification in MVP patients.
Main Methods:
- Reviewed a cardiac pathology registry of 650 young adults with SCD to identify MVP cases.
- Histopathological analysis of hearts from SCD patients with MVP.
- Contrast-enhanced cardiac magnetic resonance (CE-CMR) imaging in living MVP patients with and without complex ventricular arrhythmias.
Main Results:
- MVP was the sole cause of SCD in 7% of young adults, particularly women (13%).
- Histology revealed left ventricular fibrosis in papillary muscles and inferobasal walls in SCD patients with MVP.
- CE-CMR showed significant late gadolinium enhancement in MVP patients with arrhythmias (93%) compared to controls (14%), with fibrosis patterns overlapping histopathology.
Conclusions:
- MVP is an underrecognized cause of arrhythmic SCD, especially in young women.
- Left ventricular fibrosis, particularly in the papillary muscles and inferobasal wall, is the structural hallmark of MVP associated with ventricular arrhythmias.
- CE-CMR can identify this concealed substrate for improved risk stratification in MVP patients.
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