Incomplete double aortic arch

Case contributed by Dr Najibullah Rasouly


One-year-old boy presented with a history of cough, cyanosis and vomiting.

Patient Data

Age: 1 year
Gender: Male

A right-sided aortic arch from which the right common carotid and right subclavian arteries originate is demonstrated. The right vertebral artery arises directly from the right aortic arch between the right common carotid and right subclavian arteries. 

There is also a smaller incomplete left aortic arch from which the left common carotid and left subclavian arteries originate. The left vertebral artery also arises directly from the left aortic arch. The patent segment of the left aortic arch courses towards a diverticulum (red arrow) at the descending aorta suggesting the presence of a fibrous cord (representing an atretic segment of the left aortic arch) between the two structures. The descending aortic diverticulum is posterior to the esophagus. The esophagus proximal to the diverticulum is dilated likely due to compression by the formed vascular ring.

No other significant intra- or extracardiac congenital cardiovascular anomaly is seen.

Case Discussion

An incomplete double aortic arch with distal left arch atresia is a rare vascular ring anomaly. It can easily be mistaken for a right aortic arch with a ductus diverticulum. The posterior course of the anterior left arch towards the descending aortic diverticulum is a clue that suggests the presence of a fibrous cord (also called retroesophageal ligamentum arteriosum) which represents the atretic segment of the left arch (in contrast to an anterior course of an innominate artery in a right-sided aortic arch with mirror image branching). A prominent aortic diverticulum as in this case is difficult to diagnose on chest radiographs, although sometimes it can be seen bulging into the aortopulmonary window on lateral radiographs. Contrast-enhanced CT angiography with different image reconstruction can make the diagnosis. This anomaly is a form of vascular ring which can cause dysphagia.

Case contributed by: Dr. Najibullah Rasouly and Dr. Hidayatullah Hamidi

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Case information

rID: 52022
Published: 6th May 2017
Last edited: 6th May 2017
System: Vascular
Inclusion in quiz mode: Included

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