We present a very rare case of right aortic arch with an isolated left brachiocephalic artery in a 35-year-old female. This entity is an extremely uncommon aortic arch anomaly which has associated multisystem symptoms. We briefly discuss the hypothetical double aortic arch model originally described by Edwards. The aortic embryology can explain the anatomic findings and imaging manifestations of the isolated left brachiocephalic artery. Multimodality imaging evaluation can be helpful in detection and categorization of aortic arch abnormalities. A diagnostic imaging approach should focus on elucidating the abnormal aortic arch and great vessels arrangement with determination of associated cardiac, vascular or visceral congenital abnormalities.
Keywords: Aortic arch anomalies; CT; Isolated left brachiocephalic artery; Mirror-image branching; Nonmirror image branching; Right aortic arch.
Published by Elsevier Inc. on behalf of University of Washington.