Structure and function of the aorta in inherited and congenital heart disease and the role of MRI

1 2 In prototypical diseases such as Marfan syndrome and BAV disease, loss of fibrillin-1 microfibrils has been reported to dissociate smooth muscle cells from the medial matrix components, resulting in accelerated cell death and matrix disruption. 3 Matrix metalloproteinases (MMPs)-endogenous enzym...

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