Familial thoracic aortic aneurysm is an autosomal dominant[1] disorder of large arteries.
There is an association between familial thoracic aortic aneurysm and Marfan syndrome as well as other hereditary connective tissue disorders.
A degenerative breakdown of collagen, elastin, and smooth muscle caused by aging contributes to weakening of the wall of the artery.[2]
In the aorta, this can result in the formation of a fusiform aneurysm. There is also increased risk of aortic dissection.[citation needed]
Types include:
It is sometimes called "Erdheim cystic medial necrosis of aorta", after Jakob Erdheim.[3][4]
The term "cystic medial degeneration" is sometimes used instead of "cystic medial necrosis", because necrosis is not always found.[citation needed]