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Pathological Background

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Aortopathy

Abstract

A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and paracoarctation medial abnormalities. Congenital heart diseases (CHDs) such as single ventricle, truncus arteriosus, transposition of the great arteries, and tetralogy of Fallot are also associated with aortic medial abnormalities. A significant subset of adults with these CHD exhibits progressive dilatation of the aortic root even after repair owing to medial abnormalities that may lead to regurgitation and predispose to dissection and rupture that can be fatal and necessitating aortic valve and aortic root surgery.

Aortic medial abnormalities—incorrectly called “cystic medial necrosis”—reach their severest form in Marfan syndrome and are prevalent and qualitatively similar in a variety of CHDs with a wide age range. In Marfan syndrome, impairment of aortic medial elastic fibers is apparently more extensive than in CHD. Accordingly, the incidence of ascending aortic dilatation, dissection, or rupture is lower, and the degree of aortic root medial lesions is smaller in congenital heart disease patients with aortic dilatation. These aortic abnormalities in CHD with aortic dilatation. These aortic abnormalities in CHD are either from intrinsic factors or result from volume overload of the aorta due to right to left shunting.

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Authors and Affiliations

  1. Cardiovascular Center, St Luke’s International Hospital, 9-1 Akashi-cho, Tokyo, 104-8560, Japan

    Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC

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  1. Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC
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Correspondence to Koichiro Niwa M.D., Ph.D., FACC, FAHA, FJCC .

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Editors and Affiliations

  1. Cardiovascular Center, St Luke’s International Hospital, Chuo-ku, Tokyo, Japan

    Koichiro Niwa

  2. ACHD Unit, German Heart Centre Munich, Munich, Bayern, Germany

    Harald Kaemmerer

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Niwa, K. (2017). Pathological Background. In: Niwa, K., Kaemmerer, H. (eds) Aortopathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56071-5_2

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  • DOI: https://doi.org/10.1007/978-4-431-56071-5_2

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