Double aortic arch

Double aortic arch is the most common symptomatic type of the aortic arch variant. It may account for up to 50-60% of vascular rings.

Double aortic arch is mostly diagnosed in childhood due to of symptoms related to oesophageal and/or tracheal obstruction. Respiratory symptoms can be more common in infancy or early childhood while adult patients complain of difficulties in swallowing rather than respiratory difficulties due to tracheal development.

Double aortic arch is formed by formation of a vascular ring from the splitting of the ascending aorta into two limbs that pass to either side of the trachea and oesophagus 5 (both of which get encircled), which then join as a single descending aorta

When the minor arch is markedly hypoplastic or atretic (incomplete double aortic arch), the affected segment tends to be almost always distal to the left subclavian artery 9.

  • right dominant arch: ~75-80%
  • co-dominant arch: ~5%
  • left dominant arch: ~25%

It is rarely associated with congenital heart disease 12-13.

Right-sided aortic arch indenting the trachea and increase right paratracheal soft tissue thickness. Lack of air column in thoracic portion of the trachea sometimes seen.

Contrast swallow studies are more helpful and demonstrate classic "reverse S" indentation of the contrast column on frontal view as well as posterior indentation on lateral view. 

Contrast-enhanced cross-sectional imaging is required to confirm the diagnosis and also to delineate details of anatomy in particular right or left arch dominance for surgical planning.

Surgical intervention with tracheostomy from the side of descending aorta and division of the minor (smaller) arch and ligamentum arteriosum is indicated for symptomatic patients. In appropriate cases, surgical planning is directed toward transection of either the smallest arch (if both arches are patent) or an atretic arch segment.

Thoracic anatomy
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Article information

rID: 25605
Systems: Vascular, Chest
Section: Anatomy
Tags: variant, aorta
Synonyms or Alternate Spellings:
  • Double arch of aorta
  • Double aortic arch (DAA)
  • Double arch aorta

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