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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.

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