Magnetic resonance imaging of a distorted left subclavian artery course: An important clue to an unusual type of double aortic arch

The objective of this study was to determine if distortion of the left subclavian artery course distinguishes double aortic arch with atretic left dorsal aorta from right aortic arch with mirror image branching. We performed a retrospective case series at a tertiary care center. Twenty-six patients undergoing magnetic resonance imaging for suspicion of a vascular ring were identified, 6 of whom had an atretic left dorsal aorta confirmed by surgical inspection. Six patients with the diagnosis of right aortic arch with mirror image branching were identified for comparison. The course of the left subclavian artery was assessed using surface-rendered magnetic resonance angiography (MRA) and axial fast spin echo images. All patients with double aortic arch had clinical symptoms suggestive of esophageal or tracheal compression. Six patients had double aortic arch, 3 of whom had an atretic left dorsal aorta. In these 3 patients, the branching patterns on MRA mimicked right aortic arch mirror image branching except for the distortion of the initial course of the left subclavian artery. Surgical observation confirmed the presence of an atretic left dorsal aorta that resulted in tension on the left subclavian artery pulling it posteriorly and inferiorly and completing the vascular ring. Patients with right aortic arch mirror image branching demonstrated no such subclavian artery distortion, and these patients did not have clinical symptoms suggestive of a vascular ring. Our results demonstrate that left subclavian artery distortion due to traction by an atretic left arch is an important diagnostic finding in the evaluation 6 patients with suspected vascular rings.