Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death
Top Cited Papers
- 18 August 2015
- journal article
- research article
- Published by Ovid Technologies (Wolters Kluwer Health) in Circulation
- Vol. 132 (7), 556-566
- https://doi.org/10.1161/circulationaha.115.016291
Abstract
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.This publication has 46 references indexed in Scilit:
- Mitral valve prolapse and the mitral valve prolapse syndrome: A diagnostic classification and pathogenesis of symptomsAmerican Heart Journal, 1989
- Identification of High-Risk and Low-Risk Subgroups of Patients with Mitral-Valve ProlapseThe New England Journal of Medicine, 1989
- Arrhythmias and sudden death in mitral valve prolapseAmerican Heart Journal, 1987
- Diagnosis and classification of severity of mitral valve prolapse: Methodologic, biologic, and prognostic considerationsAmerican Heart Journal, 1987
- Echocardiographically Documented Mitral-Valve ProlapseThe New England Journal of Medicine, 1985
- Sudden death in primary mitral valve prolapseAmerican Heart Journal, 1984
- The myxomatous mitral valve and sudden death.Circulation, 1983
- The conduction system in mitral valve prolapse syndrome with sudden deathAmerican Heart Journal, 1981
- Ventricular buckling: A factor in the abnormal ventriculogram and peculiar hemodynamics associated with mitral valve prolapseAmerican Heart Journal, 1977
- Aneurysmal protrusion of the posterior leaflet of the mitral valve: An auscultatory-electrocardiographic syndromeAmerican Heart Journal, 1966