Circumflex Retroesophageal Aorta Mimicking Aortic Interruption

Abstract

Critical obstruction of the aorta presenting in the neonate typically occurs with aortic interruption or coarctation, following ductus arteriosus closure. Circumflex retroesophageal aortic arch is a rare aortic arch anomaly in which a right aortic arch continues as a retroesophageal component and descends on the left of the spine. Obstruction within the arch can occur, usually identified incidentally in older children. We report a neonate with circumflex aorta with ventricular septal defect in which there was hypoplasia of the retroesophageal segment. The patient presented with cardiovascular collapse following duct closure mimicking aortic interruption. The case is presented, together with the surgical repair.

Vascular ring associated with d-transposition of the great arteries: when should we suspect aortic arch anomalies?

“…Circumflex aortic arch is occasionally associated with hypoplasia of a retro-oesophageal segment, in which surgical reconstruction of the aortic arch is required. 7,8 Although initial echocardiographic findings mimicked coarctation of the aorta, we considered that the circumflex aortic arch was not hypoplastic in this patient. Furthermore, a case of circumflex aortic arch associated with double aortic arch has also been reported.…”

Circumflex aorta with double aortic arch in an infant

“…This pattern creates a vascular ring formed by the right arch to the right, its retroesophageal segment posteriorly, the ductus arteriosus to the left, and the pulmonary arterial bifurcation anteriorly . Compression of the esophagus and trachea results in arterial hypoplasia and obstruction . The gold standard imaging modality for this disease entity is a CT angiogram of the chest.…”

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

“…Aortic arch obstruction with a hypoplastic retroesophageal segment is the most important associated lesion. The retroesophageal arch segment is usually of adequate caliber, but significant hypoplasia can occur particularly with concomitant ventricular septal defect, which can mimic interruption [6,28,29] . When arch hypoplasia is present, it can be tubular with a long segment, or tortuous, extending from the proximal to distal transverse arch as it courses around the side of the trachea and back of the oesophagus [5][6][7]28] .…”

“…The retroesophageal arch segment is usually of adequate caliber, but significant hypoplasia can occur particularly with concomitant ventricular septal defect, which can mimic interruption [6,28,29] . When arch hypoplasia is present, it can be tubular with a long segment, or tortuous, extending from the proximal to distal transverse arch as it courses around the side of the trachea and back of the oesophagus [5][6][7]28] . A long hypoplastic retroesophageal segment can be mistaken as an Abbott artery which is an anomalous artery arising from the posterior wall of the aortic arch or others in coarctation of the aorta [30][31][32] .…”

“…Simple vascular ring division may not fully address all the deleterious effects of circumflex aorta, and a major aortic arch uncrossing procedure may be required [1][2][3][4] . The aortic arch is often unobstructed or presents with variable degrees of obstruction from hypoplasia to near-interruption requiring major arch repair [5][6][7] . When there is also obstruction of the proximal aberrant subclavian, it can be confused clinically with aortic dissection in adult patients [8] .…”