Comparison of cardiac findings in patients with mitral valve prolapse who die suddenly to those who have congestive heart failure from mitral regurgitation and to those with fatal noncardiac conditions

Farb, Andrew; Tang, Anita L.; Atkinson, James B.; McCarthy, William F.; Virmani, Renu

doi:10.1016/0002-9149(92)91281-8

Cited by 73 publications

(39 citation statements)

References 21 publications

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“…18,19 Our current study adds to the understanding of the potential characteristics of proarrhythmia in these patients. Previous studies, including Barlow original description in 1968, have reported on the association of myxomatous MVP with sudden death in the absence of severe MR. [1][2][3][4][5][6]10 Others have demonstrated the association of VA with mitral dysjunction 28 and longer mitral valve leaflets. 29 The current study compared electrophysiological study findings in patients with and without previous cardiac arrest.…”

Section: Discussionmentioning

confidence: 99%

“…A lthough there have been multiple reports of sudden unexplained death in patients with mitral valve prolapse (MVP), [1][2][3][4][5][6] prospective cohort studies have generally reported a benign prognosis in the absence of severe mitral regurgitation (MR). 7,8 Recently, our group described bileaflet MVP syndrome (BiMVPS), which seems to be associated with an increased risk of sudden death.…”

mentioning

confidence: 99%

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Sites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse Syndrome

Syed

Ackerman

McLeod

et al. 2016

Circ: Arrhythmia and Electrophysiology

122

138

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Sites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse Syndrome

Syed

Ackerman

McLeod

et al. 2016

Circ: Arrhythmia and Electrophysiology

122

138

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show abstract

“…8,[14][15][16][17] Surprisingly, no investigation systematically addressed the LV myocardium to search for the substrate of electric instability except for few anecdotic cases. 11,13,14,44,45 For the first time, we extended the histopathological investigation beyond the valve in all SCD cases and provided convincing evidence of fibrosis in the LV myocardium, which is closely linked to the mitral valve, that is, the PMs with adjacent free wall and the inferobasal wall.…”

Section: 43mentioning

confidence: 99%

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

et al. 2015

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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.

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“…Ventricular fibrillation appears to be the cause of sudden death. β-Blockade therapy may be beneficial in patients with FMV/MVP who were successfully resuscitated from cardiac arrest [1,2,52,53,54,55,56]. …”

Section: Classificationmentioning

confidence: 99%

Floppy Mitral Valve (FMV)/Mitral Valve Prolapse (MVP) and the FMV/MVP Syndrome: Pathophysiologic Mechanisms and Pathogenesis of Symptoms

Boudoulas¹,

Boudoulas

2013

Cardiology

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Mitral valve prolapse (MVP) results from the systolic movement of a portion or segments of the mitral valve leaflets into the left atrium during left ventricular systole. It is well appreciated today that floppy mitral valve (FMV) is the central issue in the MVP and mitral valve regurgitation (MVR) story. The term FMV refers to the expansion of the area of the mitral valve leaflets with elongated chordae tendineae, chordae rupture and mitral annular dilation. FMV/MVP occurs in a heterogeneous group of patients with a wide spectrum of mitral valve involvement from mild to severe. Two types of symptoms can be defined in FMV/MVP patients. In one group of patients, symptoms are directly related to progressive MVR. In the other group, symptoms cannot be explained by the degree of MVR alone; activation of the autonomic nervous system has been implicated for the explanation of symptoms in this group of patients which is referred to as the FMV/MVP syndrome. In this brief review, the natural history, pathophysiologic mechanisms and management of patients with FMV/MVP/MVR and FMV/MVP syndrome are discussed.

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Comparison of cardiac findings in patients with mitral valve prolapse who die suddenly to those who have congestive heart failure from mitral regurgitation and to those with fatal noncardiac conditions

Cited by 73 publications

References 21 publications

Sites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse Syndrome

Sites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse Syndrome

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

Floppy Mitral Valve (FMV)/Mitral Valve Prolapse (MVP) and the FMV/MVP Syndrome: Pathophysiologic Mechanisms and Pathogenesis of Symptoms

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