Altered collagen composition in a prolapsing mitral valve with ruptured chordae tendineae

Hammer, Donald; Leier, Carl V.; Baba, Nobuhisa; Vasko, John S.; Wooley, Charles F.; Pinnell, Sheldon R.

doi:10.1016/0002-9343(79)90746-0

Cited by 73 publications

(26 citation statements)

References 12 publications

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“…This classic prospective study also emphasized the broad clinical and pathologic spectrum of this form of mitral valve disease. The recent paper of Hammer et al 49 demonstrating the absence of type III collagen in a myxomatous mitral valve leaflet also lends support to the primacy of collagen abnormality.…”

Section: Discussionmentioning

confidence: 81%

"Myxomatous" mitral valves: collagen dissolution as the primary defect.

King¹,

Clark²,

Baba³

et al. 1982

Circulation

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Section: Discussionmentioning

confidence: 81%

"Myxomatous" mitral valves: collagen dissolution as the primary defect.

King¹,

Clark²,

Baba³

et al. 1982

Circulation

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“…33) In another study, the concordance between inadequate production of type III collagen and echocardiographic findings of MVP in patients with type IV Ehler-Danlos syndrome have suggested that this collagen abnormality may be the responsible factor in patients with this syndrome. 34) Abnormalities of collagen have been found in myxomatous or floppy valves of patients with MVP [11][12][13] that coincide with those identified in skin biopsies of patients with hypermobility syndrome 9) leading to the suggestion of a common pathogenetic mechanism of abnormal production or maturation of collagen. 14) Several clinical observations have led to the speculation that primary MVP syndrome represents a generalized disorder of connective tissue.…”

Section: )mentioning

confidence: 95%

“…6) In the following years, a high incidence of MVP has been reported in patients with benign joint hypermobility syndrome (BJHMS), 7,8) which, like MVP, is inherited mainly as a sex-influenced dominant trait. 9,10) Abnormalities of collagen have been found in myxomatous or floppy valves of patients with MVP [11][12][13] that coincide with those identified in skin biopsies of patients with hypermobility syndrome 9) leading to the suggestion of a common pathogenetic mechanism of abnormal production or maturation of collagen. 14) However, there have been a few studies on the relation between the echocardiographic features of mitral valves [anterior mitral leaflet thickness (AMLT), 15) maximal leaflet displacement (MLD), 7) degree of mitral regurgitation (DMR)], elastic properties of the aortic wall [index of aortic systolic (AOSDI) and diastolic diameters (AODDI), index of aortic stiffness (IAOS) and aortic distensibility (AOD)], and Beighton hypermobility score (BHS) in MVP patients with BJHMS.…”

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confidence: 95%

The Relationship Between Echocardiographic Features of Mitral Valve and Elastic Properties of Aortic Wall and Beighton Hypermobility Score in Patients With Mitral Valve Prolapse

et al. 2004

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SUMMARYThe present study was designed to investigate the incidence of benign joint hypermobility syndrome (BJHMS) in mitral valve prolapse (MVP) and the correlation between the echocardiographic features of the mitral valve and elastic properties of the aortic wall and Beighton hypermobility score (BHS) in patients with MVP and BJHMS.Fourty-six patients with nonrheumatic, uncomplicated, and isolated mitral anterior leaflet prolapse (7 men and 39 women, mean age; 26.1 ± 5.9) and 25 healthy subjects (3 men and 22 women, mean age, 25.4 ± 4.3) were studied. Patients were divided into two groups according to their BHS (group I, MVP+BJHMS; group II, MVP-BJHMS). Individuals with accompanying cardiac or systemic disease were excluded. Echocardiographic examination was performed in all subjects. The presence of BJHMS was evaluated according to Beighton's criteria.The incidence of BJHMS in patients with MVP was found to be significantly higher than that of controls (45.6%, (21/46) vs 12% (3/25), P < 0.0001). Group I (MVP + BJHMS) had significantly increased anterior mitral leaflet thickness (AMLT, 3.4 ± 0.4 vs 3.1 ± 0.3; P < 0.005), maximal leaflet displacement (MLD, 2.4 ± 0.4 vs 1.7 ± 0.4; P < 0.005), and degree of mitral regurgitation (DMR, 17.1 ± 7.2 vs 11.2 ± 4.4; P < 0.01) compared to group II. However, the index of aortic stiffness (IAOS) was found to be lower (17.6 ± 6.9 vs 23.9 ± 7.6; P < 0.005) and aortic distensibility (AOD) to be higher (0.0035 ± 0.007 vs 0.0024 ± 0.005; P < 0.005) in group I. There was a significant correlation between AMLT, MLD and DMR, and BHS (r = 0.57/P = 0.007, r = 0.55/P < 0.009, r = 0.51/P < 0.01, respectively). In addition, AOD correlated positively with BHS (r = 0.53/ P < 0.005), but the index of aortic stiffness correlated inversely with BHS (r = -0.49/P < 0.007).The incidence of BJHMS in patients with MVP was more frequent than the normal population and there was a significant correlation between the severity of BJHMS From the

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“…[1][2][3][4][5] The disease involves progressive myxomatous degeneration of the mitral valve and chordae tendineae and has close pathologic similarities to myxomatous degeneration of the mitral valve in humans. [6][7][8] Myxomatous degeneration results in disturbance of collagen deposition and organization and increased amounts of acid mucopolysaccharides. The gross and histopathologic appearance of affected valve leaflets in dogs has been reported.…”

mentioning

confidence: 99%

Ultrastructural morphologic evaluation of the phenotype of valvular interstitial cells in dogs with myxomatous degeneration of the mitral valve

Black

French²,

Dukes‐McEwan³

et al. 2005

ajvr

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Myxomatous degeneration of the mitral valve in dogs is associated with phenotypic alteration, changing from an interstitial to a mixed myofibroblast or smooth muscle cell phenotype. A closer association between interstitial cells and the endothelium is evident in diseased valves. In response to the disease process, valvular interstitial cells of dogs appear to change toward a smooth muscle phenotype, possibly in an attempt to maintain valve tone and mechanical function.

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Altered collagen composition in a prolapsing mitral valve with ruptured chordae tendineae

Cited by 73 publications

References 12 publications

"Myxomatous" mitral valves: collagen dissolution as the primary defect.

"Myxomatous" mitral valves: collagen dissolution as the primary defect.

The Relationship Between Echocardiographic Features of Mitral Valve and Elastic Properties of Aortic Wall and Beighton Hypermobility Score in Patients With Mitral Valve Prolapse

Ultrastructural morphologic evaluation of the phenotype of valvular interstitial cells in dogs with myxomatous degeneration of the mitral valve

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