Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8657684 | Cardiovascular Pathology | 2018 | 8 Pages |
Abstract
Pathogenesis of aortopathy in MFS overlaps with mechanisms seen in BAV and TAV, leading to a so called double hit hypothesis for aortic complications in MFS. The ascending aortic wall in MFS is immature with undifferentiated VSMCs and low levels of fibrillin-1. The immature media becomes even more vulnerable for aortopathy due to other degenerative features which develop probably as a direct consequence of the fibrillin-1 mutation.
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Authors
Nimrat Grewal, Adriana C. Gittenberger-de Groot,