Pharmacotherapy in Aortic Disease by Arturo Evangelista & Christoph A. Nienaber

Author:Arturo Evangelista & Christoph A. Nienaber
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

(b) MYH11 gene mutations are responsible for familial forms of TAA often with patent ductus arteriosus [17]. They are very rare.

Dilatation of the aorta is mostly located at the level of the sinuses of Valsalva, and usually is symmetrical (Fig. 3.3). In some aetiologies (ACTA2, MYH11), dilatation can also be observed in the tubular portion of the ascending aorta.

Bicuspid Aortic Valve

Some TAA have been associated with a bicuspid aortic valve (BAV), consequently the frequency of TAA is greater in patients with BAV than in the general population. The reasons for this association are unclear, and 3 main hypothesis can be proposed [18]:Aortic root dilatation may be constitutive, i.e. not reflecting a progressive increase in diameter with time. This may be close to the abnormal aortic cusps i.e. at the level of the sinuses of Valsalva, but not above the sino-tubular junction.

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