2004
DOI: 10.1172/jci23701
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Marfan syndrome and mitral valve prolapse

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Cited by 42 publications
(8 citation statements)
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“…In the mouse model studied in this report, enhanced TGF-β-mediated signals were a consequence of fibrillin-1 deficiency. As pointed out by Weyman and Scherrer-Crosbie [4], such abnormalities have not been demonstrated in sporadic MVP patients or families with the disorder. Other discrepancies of this model with the clinical presentation of MVP include adult onset compared with onset in infancy in Marfan syndrome, and homogeneous valve involvement with extensive valvular interstitial cell proliferation in the latter [3].…”
Section: Discussionmentioning
confidence: 98%
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“…In the mouse model studied in this report, enhanced TGF-β-mediated signals were a consequence of fibrillin-1 deficiency. As pointed out by Weyman and Scherrer-Crosbie [4], such abnormalities have not been demonstrated in sporadic MVP patients or families with the disorder. Other discrepancies of this model with the clinical presentation of MVP include adult onset compared with onset in infancy in Marfan syndrome, and homogeneous valve involvement with extensive valvular interstitial cell proliferation in the latter [3].…”
Section: Discussionmentioning
confidence: 98%
“…While inherited connective tissue disorders, such as Marfan syndrome, have provided potential mechanistic insights into the pathogenesis of this disorder, the majority of MVP cases are sporadic. Furthermore, the murine fibrillin-1 knockout model of Marfan syndrome bears only a partial resemblance to human MVP [3,4]. …”
Section: Introductionmentioning
confidence: 99%
“…These findings align closely with our results, which suggest that the functional consequence of valve thickening, reduced fiber alignment, and abnormal matrix deposition ultimately leads to a reduction in tissue stiffness. Upon substantial remodeling, the functional consequence of MV leaflets can ultimately result in prolapse and regurgitation [36] . Although several studies have reported disparate prevalence of MV prolapse among individuals with MFS, the biomechanical remodeling within the MV is substantial [37] .…”
Section: Discussionmentioning
confidence: 99%
“…However, all the selected MVP patients had only posterior MVP. It is known that two-thirds of MVP patients present the posterior prolapse, while the remaining third show anterior or bileaflet prolapse [ 22 , 23 ]. Considering this, to obtain a model able to discriminate MVP from healthy subjects, in primary screening, we must use a population with not only posterior but also anterior and bileaflet prolapse.…”
Section: Discussionmentioning
confidence: 99%