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Landmark lecture: Perloff lecture: Tribute to Professor Joseph Kayle Perloff and lessons learned from him: aortopathy in adults with CHD*

Published online by Cambridge University Press:  29 December 2017

Koichiro Niwa
Koichiro Niwa*
Affiliation:
Department of Cardiology, Cardiovascular Center, St Luke’s International Hospital, Tokyo, Japan
*
Correspondence to: K. Niwa, MD, PhD, FAHA, FACC, FJCC, Department of Cardiology, Cardiovascular Center, St luke’s International Hospital, Tokyo 1048560, Japan. Tel: 81335415151; Fax: 81355507194; E-mail: kniwa@aol.com
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Abstract

Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatation and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, a new clinical entity can be used to call this association of aortic pathophysiological abnormality, aortic dilation, and aorto-left ventricular interaction – “aortopathy”.

Keywords

Aortic dilatationaortopathyJoseph Perloffcystic medial necrosisaortic elasticity
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Special Issue 10: Proceedings of the 2017 7th World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS 2017) Barcelona, Spain July 16–21, 2017 , December 2017 , pp. 1959 - 1965
Copyright
© Cambridge University Press 2017 

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Footnotes

*

Presented at the 2017 Seventh World Congress of Pediatric Cardiology & Cardiac Surgery (WCPCCS 2017), Barcelona, Spain, 16–21 July, 2017. Presented Friday, 21 July, 2017.

†

Presidents of the Japanese Society for Adult Congenital Heart Disease and Asia Pacific Society for Adult Congenital Heart Disease.

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