Sudden death in the mitral valve prolapse-click syndrome

Jeresaty, Robert M.

doi:10.1016/0002-9149(76)90328-3

Cited by 131 publications

(23 citation statements)

References 8 publications

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“…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. The LV myocardial scarring is qualitatively different from that observed in ischemic heart disease, where it is usually compact and confluent, instead being patchy and interspersed within surviving, hypertrophic cardiomyocytes.…”

Section: 43mentioning

confidence: 81%

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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Section: 43mentioning

confidence: 81%

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

et al. 2015

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“…This new appreciation should enhance our ability to ask appropriate clinical questions relating mitral valve shape and leaflet displacement to clinical and pathologic consequences. (Circulation 1989;80:589-598) irat valve prolapse, initially believed to be uncommon,' has evolved into a pervasive clinical problem.2'3 Based on a variety of criteria, it can be diagnosed in a disturbingly high proportion of individuals from the general population, many of whom are otherwise normal2 -8 but who are nevertheless given an uncertain prognosis that includes endocarditis, stroke, and sudden death.1, [9][10][11] To resolve this confusion requires a return to basic definitions. Prolapse is the displacement of a bodily part from its normal position or relations.…”

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confidence: 99%

Three-dimensional echocardiographic reconstruction of the mitral valve, with implications for the diagnosis of mitral valve prolapse.

et al. 1989

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Mitral valve prolapse has been diagnosed by two-dimensional echocardiographic criteria with surprising frequency in the general population, even when preselected normal subjects are examined. In most of these individuals, however, prolapse appears in the apical four-chamber view and is absent in roughly orthogonal long-axis views. Previous studies of in vitro models with nonplanar rings have shown that systolic mitral annular nonplanarity can potentially produce this discrepancy. However, to prove directly that apparent leaflet displacement in a two-dimensional view does not constitute true displacement above the three-dimensional annulus requires reconstruction of the entire mitral valve, including leaflets and annulus. Such reconstruction would also be necessary to explore the complex geometry of the valve and to derive volumetric measures of superior leaflet displacement. A technique was therefore developed and validated in vitro for three-dimensional reconstruction of the entire mitral valve. In this technique, simultaneous real-time acquisition of images and their spatial locations permits reconstruction of a localized structure by minimizing the effects of patient motion and respiration. By applying this method to 15 normal subjects, a coherent mitral valve surface could be reconstructed from intersecting scans. The results confirm mitral annular nonplanarity in systole, with a maximum deviation of 1.490.3 cm from planarity. They directly show that leaflets can appear to ascend above the mitral annulus in the apical four-chamber view, as they did in at least one view in all subjects, without actual leaflet displacement above the entire mitral valve in three dimensions, thereby challenging the diagnosis of prolapse by isolated fourchamber view displacement in otherwise normal individuals. This technique allows us to address a uniquely three-dimensional problem with high resolution and provide new information previously unavailable from the two-dimensional images. This new appreciation should enhance our ability to ask appropriate clinical questions relating mitral valve shape and leaflet displacement to clinical and pathologic consequences. (Circulation 1989;80:589-598) irat valve prolapse, initially believed to be uncommon,' has evolved into a pervasive clinical problem.2'3 Based on a variety of criteria, it can be diagnosed in a disturbingly high proportion of individuals from the general population, many of whom are otherwise normal2-8 but who are nevertheless given an uncertain prognosis that includes endocarditis, stroke, and sudden death.1,9-11To resolve this confusion requires a return to basic definitions. Prolapse is the displacement of a bodily part from its normal position or relations.12

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“…It is suggested that QT prolongation is related to arrhythmic sequelae in MVP cases, 3,7,21,22 but controversies still exist. 21 Different studies present the incidence of QTc prolongation between 8 and 75% in MVP, 7,21 whereas the Framingham Study reported no relation between these entities.…”

Section: Discussionmentioning

confidence: 99%

“…9,10 In spite of the contradictory information on the relation between QT prolongation and MVP, it is focused on the link between nonspecific repolarization changes and potentially malign arrhythmia. 22 The inter-lead variability of the QT interval measured in the 12-lead ECG is defined as QTd. Mirvis 23 and others 24 showed variability in QTd and established the QTd.…”

Section: Discussionmentioning

confidence: 99%

Relation Between QT Dispersion and Ventricular Arrhythmias In Uncomplicated Isolated Mitral Valve Prolapse

et al. 1999

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itral valve prolapse (MVP) is the most commonly diagnosed valvular heart disease, especially in the young, and affects 5% of the community. 1 Although it is reported that MVP has a good prognosis and a low incidence of complications, 2 it is obvious that even a rare complication will affect many individuals due to the high incidence of MVP in the general population. 3 However, some reports point out that these complications are not as rare as was thought, 4,5 the main entities being endocarditis, cerebrovascular accidents, sudden death, ventricular tachycardia and ventricular fibrillation. Arrhythmia and sudden death are significant complications in MVP cases; a 0.5% incidence of sudden death has been reported in different MVP studies 3,6 and it is suggested that the underlying mechanism of the sudden death is arrhythmogenic. It was also claimed that the prolonged QT intervals in MVP cases could be related to arrhythmia and sudden death. 7,8 Furthermore, there are studies that found that, instead of a prolonged QT interval increase, intrinsic QT dispersion can be related to arrhythmia in these cases. 9,10 In the present study, we aimed to determine the relationship between intrinsic QT dispersion and ventricular Japanese Circulation Journal Vol.63, December 1999 arrhythmias and sudden death in young individuals suffering from uncomplicated isolated MVP (IMVP). In this respect, cases without leaflet thickening (≥5 mm), severe mitral regurgitation or left ventricular systolic dysfunction were included in the study. Although a few reports have concentrated on the clinical significance of MVP accompanying tricuspid valve prolapse (TVP), the clinical significance of TVP and its relation to the arrhythmias of MVP has not yet been clearly documented. Therefore, we excluded MVP cases with accompanying TVP after beginning the study. Our main aim was to investigate the incidence and distribution of ventricular arrhythmias, according to their severity and relation to the QT interval, and to the dispersion of repolarization in uncomplicated IMVP. Methods Study GroupsThe current study was carried out in the Department of Cardiology, Faculty of Medicine, Dicle University between May 1996 and April 1998. We registered 91 individuals who underwent echocardiographic examination and were diagnosed as MVP; the group included 33 cases with accompanying TVP. Those patients who had disorders such as ischemic or rheumatic heart disease, systemic and/or pulmonary hypertension, diabetes mellitus, hyperthyroidism, stenotic valvular heart disease, congenital heart diseases, severe mitral regurgitation (>3 cm 2 regurgitant jet length in color flow mapping), or severe left ventricular dysfunction Jpn Circ J 1999; 63: 929 -933 (Received November 16, 1998; revised manuscript received July 16, 1999; accepted July 23, 1999 Complications of mitral valve prolapse (MVP), among which serious ventricular arrhythmia and sudden death are of major importance, affect many individuals due to the high incidence of MVP itself in the community despite t...

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Sudden death in the mitral valve prolapse-click syndrome

Cited by 131 publications

References 8 publications

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

Three-dimensional echocardiographic reconstruction of the mitral valve, with implications for the diagnosis of mitral valve prolapse.

Relation Between QT Dispersion and Ventricular Arrhythmias In Uncomplicated Isolated Mitral Valve Prolapse

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