Atretic Double Aortic Arch: Imaging Appearance of a Rare Anomaly and Differentiation From Its Mimics.

Sarv Priya,Prashant Nagpal

doi:10.7759/cureus.9478

Abstract

A double aortic arch (DAA) with atresia is an uncommon cause of a symptomatic vascular ring resulting in trachea-esophageal compression. An atretic double aortic arch can resemble the right aortic arch with a mirror image branching pattern or the right arch with an aberrant left subclavian artery depending upon the level of atresia. The double aortic arch with atresia is difficult to detect on pre-surgical computed tomography angiography or magnetic resonance angiography due to a lack of contrast in the obliterated arch segment. Differentiation of a double arch with atresia from the right aortic arch is vital as an atretic double arch is a form of the complete vascular ring while the right aortic arch may or may not be symptomatic. Knowledge of some key imaging features can help distinguish these entities. In this case report, we discuss an uncommon case of a double aortic arch with atresia between the left common carotid and left subclavian artery. We also describe its close mimics, their embryological basis, and ways to differentiate it from the right aortic arch.

Highlights

Vascular rings are a rare form of aortic arch anomaly resulting in trachea-esophageal compression and represent 1%-3% of all congenital heart diseases [1]
An atretic double arch can mimic the right-sided aortic arch with mirror image branching or right arch with aberrant left subclavian artery depending upon the level of atresia
We highlight a rare case of Double aortic arch (DAA) with atresia and discuss approaches to accurately characterize the atretic double aortic arch and describe diagnostic clues to differentiate it from mimics of the right aortic arch

Summary

Introduction

Vascular rings are a rare form of aortic arch anomaly resulting in trachea-esophageal compression and represent 1%-3% of all congenital heart diseases [1]. The left subclavian artery (LSCA) was seen coursing behind the esophagus (Figure 1C) with the right-sided descending aorta (Video 1). VIDEO 1: Double aortic arch with atresia between the left common carotid and the left subclavian artery in a two-year-old male child. The presence of symmetric four-vessel aortic arch branches, the posterior course of the first aortic arch branch (LCCA), and diverticular outpouching from the descending thoracic aorta helped suggest that imaging was highly suggestive of DAA with atresia between the LCCA and LSCA. Due to the concern of complete vascular ring and symptoms, the child underwent surgical repair via left thoracotomy, and an obliterative segment was found between LCCA and LSCA, confirming the atresia as seen on CTA chest. The child was successfully treated by the division of the atretic left-sided aortic arch

Discussion

Conclusions

Disclosures

Weinberg PM