Double aortic arch is seen with the ascending aorta is seen splitting into the right arch which is the larger and more superior and the left arch which is the smaller and at a lower level. The double aortic arch forms a vascular ring with compression of the proximal esophagus posteriorly as well as abutment and slight compression of the trachea. The double aortic arches unite posteriorly and form the descending aorta.
Two brachiocephalic arteries arise separately from the right and left arches with the four vessel sign.
No otherwise cardiac anomaly.