Association of Mitral Annular Disjunction With Premature Cardiac Mortality in a Large Series of Autopsies

Zhou, Nan; Zhao, Qianhao; Zeng, Xiaofeng; Zheng, Dong-Hui; Yue, Jiacheng; Zhang, Kai; Mao, Danmi; Makielski, Jonathan C.; Cheng, Jianding

doi:10.1016/j.jacc.2020.10.051

Cited by 10 publications

(9 citation statements)

References 4 publications

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“…On pathological examination, the diagnosis of MAD is made by observing a separation between the attachment of the posterior MV leaflet to the LA wall and the LA-LV junction. 4 In the histological image of MAD originally published by Perazzolo Marra et al, 5 mitral valve leaflet tissue is observably attached to left atrial tissue at a point separate from the connection of left atrial tissue to the more muscular left ventricular tissue (Figure 2). Based on this and prior autopsy studies, MAD tissue appears to be created by a stretching or "curtain-like" deformation of the fibrous annulus, and atrial myocytes may not be present in the region of disjunction.…”

Section: Diagnosis and Imagingmentioning

confidence: 94%

“…In studies that have examined more general populations, not just those with known MVD, estimates of MAD prevalence are typically lower, ranging from 4.1% to 22.9%. 2,4,21 However, in a recent review of 98 subjects with structurally normal hearts who underwent CCT, Toh et al 11 reported that 96% had MAD. No minimum distance was used to define MAD positivity, and the median MAD distance was just 3.0 mm with a range of 1.5 to 7 mm.…”

Section: Prevalencementioning

confidence: 99%

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Imaging Considerations and Clinical Implications of Mitral Annular Disjunction

Drescher

Kelsey

Yankey

et al. 2022

Circ: Cardiovascular Imaging

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Mitral annular disjunction is increasingly recognized as an important anatomic feature of mitral valve disease. The presence of mitral annular disjunction, defined as separation between the left atrial wall at the point of mitral valve insertion and the left ventricular free wall, has been associated with increased degeneration of the mitral valve and increased incidence of sudden cardiac death. The clinical importance of this entity necessitates standard reporting on cardiovascular imaging reports if patients are to receive adequate risk stratification and management. We provide a narrative review of the literature pertaining to mitral annular disjunction, its clinical implications, and areas needing further research.

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Section: Diagnosis and Imagingmentioning

confidence: 94%

Section: Prevalencementioning

confidence: 99%

Imaging Considerations and Clinical Implications of Mitral Annular Disjunction

Drescher

Kelsey

Yankey

et al. 2022

Circ: Cardiovascular Imaging

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“…23,24 However, it is also seen in ischaemic heart disease and in those without MVP. 23,25 Myocardial fibrosis has been suggested as a substrate for ventricular arrhythmias at both autopsy 10,24 and imaging. 26 Our study confirms ventricular fibrosis in a vast majority of cases (90%), so magnetic resonance imaging (MRI) of the heart in the living may add to the risk profile for SCD.…”

Section: Discussionmentioning

confidence: 99%

Insights into malignant mitral valve degenerative disease from a sudden cardiac death cohort highlighting significant measurement differences from normal

Westaby,

Bicalho,

Zullo

et al. 2024

Histopathology

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AimsMitral valve prolapse (MVP) is an accepted cause of sudden cardiac death (SCD) in most autopsy series. Diagnosis at autopsy relies upon subjective assessment with no established objective pathological criteria. This study set out to establish objective measurements to help pathologists dealing with SCD.MethodsWe diagnosed 120 (1.5%) cases of MVP in 8108 cases of SCD. We measured the mitral annulus, anterior and posterior leaflets, rough zone and mitral annular disjunction (MAD) in 27 MVP cases and compared them to 54 age‐ and sex‐matched normal mitral valves.ResultsAge of death was 39 ± 16 years, with 59 females and 61 males. History of mild MV disease was present in 19 (16%). Eleven (9%) died associated with exertion. Left ventricular hypertrophy was present in nine (15%) females and 10 (16%) males. Both MV leaflets showed thickening and ballooning in all individuals. MVP showed highly significantly increased annular circumference, elongation and thickening of both leaflets as well as increased MAD (all P < 0.001). Left ventricular fibrosis was present in 108 (90%), with interstitial fibrosis in the posterolateral wall and papillary muscle in 88 (81%) and coexisting replacement fibrosis in 40 (37%).ConclusionThis is the largest MVP associated with SCD series highlighting a young cohort with equal representation of males and females. There is involvement of both leaflets with significant annular dilatation, elongation and thickening of both leaflets with MAD. Left ventricular fibrosis explains arrhythmia. Our quantitative measurements should serve as a reference for pathologists assessing post‐mortem hearts for MVP.

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“…Risk factors associated with malignant arrhythmias include palpitations, certain causative fibrillin-1 mutations, MVP, left ventricular dilation, dysfunction, and repolarization abnormalities ( 2 , 3 , 4 , 5 ). MAD has hitherto not been described in pediatric patients, but growing evidence suggests a relationship between MAD and ventricular arrhythmias and sudden cardiac death (SCD) ( 6 , 7 ). MAD is abnormal atrial displacement of the mitral hinge point, and it usually, albeit not always, appears with concomitant mitral valve disease, including MVP and myxomatous (or Barlow’s) mitral valve, both of which are common in patients with MFS.…”

Section: Discussionmentioning

confidence: 99%

Mitral Annular Disjunction Associated With Fatal Ventricular Arrhythmia in an Adolescent With Marfan Syndrome

2021

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We discuss an adolescent with Marfan syndrome and a previous aortic valve-sparing root replacement who was found to have mitral annular disjunction on surveillance cardiac imaging in the setting of recurrent palpitations. Ambulatory heart rate monitoring incidentally captured a fatal ventricular arrhythmia, a well-recognized but underappreciated cause of sudden cardiac death in patients with Marfan syndrome. ( Level of Difficulty: Advanced. )

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Association of Mitral Annular Disjunction With Premature Cardiac Mortality in a Large Series of Autopsies

Cited by 10 publications

References 4 publications

Imaging Considerations and Clinical Implications of Mitral Annular Disjunction

Imaging Considerations and Clinical Implications of Mitral Annular Disjunction

Insights into malignant mitral valve degenerative disease from a sudden cardiac death cohort highlighting significant measurement differences from normal

Mitral Annular Disjunction Associated With Fatal Ventricular Arrhythmia in an Adolescent With Marfan Syndrome

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