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History of Aortopathy

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Aortopathy

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Abstract

After Maude Abbott mentioned in 1928 in her atlas of congenital heart disease that the presence of a bicuspid aortic valve appears to indicate a tendency for spontaneous rupture, the following story started: recognized were a high incidence of aortic dissection and medial intrinsic abnormalities of the aorta in the presence of a bicuspid aortic valve, medial abnormalities (resembling Marfan aorta) in various congenital heart anomalies with dilated aorta, risk factors for this dilatation, reduced aortic elasticity influencing coronary artery, and ventricular performance, and finally, the concept of aortopathy in congenital heart diseases has been established.

Medial abnormalities in the ascending aorta were prevalent in a variety of congenital heart anomalies such as bicuspid aortic valve, coarctation of the aorta, single ventricle with pulmonary atresia or stenosis, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot and may predispose to dilatation and aneurysmal formation. These congenital heart diseases exhibit ongoing dilatation of the aortic root and reduced aortic elasticity that may relate to intrinsic properties of the aortic root. Aortic dilatation and increased stiffness can also induce left ventricular hypertrophy, reduced coronary artery flow, and left ventricular dysfunction. This association of aortic pathophysiological abnormality, aortic dilation, and aorto-ventricular interaction could be recognized as a new clinical entity: “aortopathy.”

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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). History of Aortopathy. In: Niwa, K., Kaemmerer, H. (eds) Aortopathy. Springer, Tokyo. https://doi.org/10.1007/978-4-431-56071-5_1

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  • DOI: https://doi.org/10.1007/978-4-431-56071-5_1

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  • Print ISBN: 978-4-431-56069-2

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