Sudden death in primary mitral valve prolapse

Pocock, Wendy A.; Bosman, C; Chesler, Elliot; Barlow, John B.; Edwards, Jesse E.

doi:10.1016/0002-8703(84)90389-2

Cited by 94 publications

(26 citation statements)

References 15 publications

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“…The association between mitral valve prolapse and sudden death has been controversial, and the increase in risk of sudden death with this condition is probably very small. [12][13][14] For the purpose of the present study, when pathological criteria for lone mitral valve prolapse were met, hearts were considered to have a nonspecific structural abnormality. 15 …”

Section: Detailed Methods Of Pathological Examinationmentioning

confidence: 99%

Sudden Cardiac Death With Apparently Normal Heart

2000

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Background-Mechanisms of sudden cardiac death (SCD) in subjects with apparently normal hearts are poorly understood. In survivors, clinical investigations may not establish normal cardiac structure with certainty. Large autopsy series may provide a unique opportunity to confirm structural normalcy of the heart before reviewing a patient's clinical history. Methods and Results-We identified and reexamined structurally normal hearts from a 13-year series of archived hearts of patients who had sudden cardiac death. Subsequently, for each patient with a structurally normal heart, a detailed review of the circumstances of death as well as clinical history was performed. Of 270 archived SCD hearts identified, 190 were male and 80 female (mean age 42 years); 256 (95%) had evidence of structural abnormalities and 14 (5%) were structurally normal. In the group with structurally normal hearts (mean age 35 years), SCD was the first manifestation of disease in 7 (50%) of the 14 cases. In 6 cases, substances were identified in serum at postmortem examination without evidence of drug overdose; 2 of these chemicals have known associations with SCD. On analysis of ECGs, preexcitation was found in 2 cases. Comorbid conditions identified were seizure disorder and obesity (2 cases each). In 6 cases, there were no identifiable conditions associated with SCD. Conclusions-In 50% of cases of SCD with structurally normal hearts, sudden death was the first manifestation of disease.An approach combining archived heart examinations with detailed review of the clinical history was effective in elucidating potential SCD mechanisms in 57% of cases. (Circulation. 2000;102:649-654.)

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Section: Detailed Methods Of Pathological Examinationmentioning

confidence: 99%

Sudden Cardiac Death With Apparently Normal Heart

2000

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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.…”

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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“…Floppy mitral valve is a recognized cause of sudden death [220][221][222][223]. The mechanism is not understood: mitral regurgitation is not a necessary factor and arrhythmia appears to be the likely mechanism [224].…”

Section: Sudden Cardiac Death: Other Aetiologiesmentioning

confidence: 99%

Sudden adult death

Langlois

2009

Forensic Sci Med Pathol

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In the investigation of sudden death in adults, channelopathies, such as long QT syndrome, have risen to the fore in the minds of forensic pathologists in recent years. Examples of these disorders are touched upon in this review as an absence of abnormal findings at postmortem examination is characteristic and the importance of considering the diagnosis lies in the heritable nature of these conditions. Typically, a diagnosis of a possible channelopathy is evoked as an explanation for a 'negative autopsy' in a case of apparent sudden natural death. However, the one potential adverse effect of this approach is that subtle causes of sudden death may be overlooked. The intention of this article is to review and discuss potential causes of sudden adult death (mostly natural) that should be considered before resorting to a diagnosis of possible channelopathy. Nonetheless, it becomes apparent that many of the potential causes of sudden death can have a genetic basis. Thus, it becomes an important consideration that there may be a genetic basis to sudden death that extends beyond the negative autopsy.

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Sudden death in primary mitral valve prolapse

Cited by 94 publications

References 15 publications

Sudden Cardiac Death With Apparently Normal Heart

Sudden Cardiac Death With Apparently Normal Heart

Arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death

Sudden adult death

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