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Bader et al. Vessel Plus 2020;4:34 Vessel Plus
DOI: 10.20517/2574-1209.2020.36

Review Open Access

Congenital arch malformation: a review of
morphology and surgical management of circumflex

aortic arch

Vivian Bader , W. Brodie Knight , Mark H. Danton , Ed Peng 1,3
1,3
1
2
1 Department of Paediatric Cardiac Surgery, Royal Hospital for Children, Glasgow, Scotland G51 4TF, United Kingdom.
2 Paediatric Cardiology department, Scottish Paediatric Cardiac Services, Royal Hospital for Children, Glasgow, Scotland G51
4TF, United Kingdom.
3 College of Medical, Veterinary and Life Sciences, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow G12
8QQ, United Kingdom.

Correspondence to: Mr. Ed Peng, Department of Paediatric Cardiac Surgery, Royal Hospital for Children, Queen Elizabeth
University Hospital, New Office Block, 1345 Govan Rd, Glasgow, Scotland G51 4TF, United Kingdom.
E-mail: edwkpeng@doctors.org.uk

How to cite this article: Bader V, Knight WB, Danton MH, Peng E. Congenital arch malformation: a review of morphology and
surgical management of circumflex aortic arch. Vessel Plus 2020;4:34. http://dx.doi.org/10.20517/2574-1209.2020.36

Received: 3 Aug 2020 First Decision: 21 Sep 2020 Revised: 28 Sep 2020 Accepted: 12 Oct 2020 Published: 13 Nov 2020
Received: First Decision: Revised: Accepted: Published: x
Academic Editor: Lizhong Sun, Junmin Zhu, Haiou Hu Copy Editor: Cai-Hong Wang Production Editor: Jing Yu
Science Editor: Copy Editor: Production Editor: Jing Yu

Abstract
Circumflex aorta is an unusual form of congenital arch malformation with a retroesophageal arch segment.
Circumflex aorta crosses the midline behind the oesophagus and above the level of carina to become continuous
with the proximal descending aorta on the opposite side to form a true vascular ring with the arterial ligament.
The term is often used to refer to the more common variant of circumflex aorta with a retroesophageal right-sided
aortic arch with a left-sided descending aorta and left-sided ligamentum. Its mirror-form with a retroesophageal
left-sided aortic arch is much rarer. Although originally described without obstruction, it may occur in association
with aortic arch hypoplasia, adding to its clinical burden. This article describes the morphology of circumflex aorta
and its clinical presentation, and provides review of surgical management of circumflex aortic arch.

Keywords: Congenital, arch malformation, morphology, circumflex aorta, surgery

INTRODUCTION
Circumflex aorta is an unusual form of congenital aortic arch anomaly with a retroesophageal arch
segment. The term is most commonly used to refer to an aorta that passes to the right of the trachea (i.e.,

© The Author(s) 2020. Open Access This article is licensed under a Creative Commons Attribution 4.0
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