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Persistent fifth aortic arch: the “great pretender” in clinical practice

Published online by Cambridge University Press:  30 August 2017

David F. A. Lloyd ,
S. Y. Ho ,
Kuberan Pushparajah ,
Subhasis Chakraborty ,
Mohamed Nasser ,
Hideki Uemura ,
Rodney Franklin  and
Alan G. Magee
David F. A. Lloyd*
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
S. Y. Ho
Affiliation:
Cardiac Morphology Unit, Royal Brompton Hospital, London, United Kingdom
Kuberan Pushparajah
Affiliation:
Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
Subhasis Chakraborty
Affiliation:
Paediatric Radiology Department, John Radcliffe Hospital, Oxford, United Kingdom
Mohamed Nasser
Affiliation:
Department of Congenital Heart Disease, Evelina Children’s Hospital, London, United Kingdom
Hideki Uemura
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
Rodney Franklin
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
Alan G. Magee
Affiliation:
Department of Congenital Heart Disease, Royal Brompton Hospital, London, United Kingdom
*
Correspondence to: Dr D. Lloyd, Department of Congenital Heart Disease, Evelina Children’s Hospital, 6th Floor, London, SE1 7TH, United Kingdom. Tel: 02071888552; E-mail: david.lloyd@gstt.nhs.uk
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Abstract

Persistence of the embryonic “fifth aortic arch” in postnatal life is a rare, enigmatic – and at times controversial – condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the “great pretender” by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of “persistent fifth aortic arch” while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

Keywords

CHDcoarctation of the aortaarterial ductpersistent fifth aortic arch
Type
Review Article
Information
Cardiology in the Young , Volume 28 , Issue 2 , February 2018 , pp. 175 - 181
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Copyright
© Cambridge University Press 2017 

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