Double aortic arch

This code describes a congenital cardiovascular anomaly where two aortic arches form a vascular ring, encircling and potentially compressing the trachea and esophagus. This malformation results from the abnormal persistence of both the right and left fourth embryonic aortic arches.

Use this code for documented cases of double aortic arch identified through imaging studies such as echocardiography, CT angiography, or MRI. It is appropriate for both symptomatic and asymptomatic presentations of this congenital heart defect.

• Ensure documentation clearly specifies "double aortic arch" and not other forms of vascular rings or isolated arch anomalies.
• Code any associated symptoms (e.g., dysphagia, stridor, recurrent respiratory infections) separately.
• Do not confuse with other aortic arch anomalies like interrupted aortic arch (Q25.21) or coarctation of the aorta (Q25.1).