Double aortic arch

Alternative Names:

Aortic arch anomaly; Double arch

Signs and tests:

The symptoms may lead a doctor to suspect double aortic arch. Other tests will then be needed to diagnose this condition.

The following tests can help diagnose double aortic arch:

Chest x-ray
Scans that create cross-sectional images of the body (CT or MRI scan)
Ultrasound examination of the heart (echocardiography)
X-ray using a substance that outlines the esophagus (barium swallow)

Treatment:

Surgery can be done to fix double aortic arch. The surgery separates off the smaller branch of the double aortic arch to relieve pressure on the esophagus and windpipe (trachea).

The surgeon ties off the smaller branch and separates it from the larger branch. Then the surgeon closes the ends of the aorta with stitches.

Expectations (prognosis):

Surgery can treat double aortic arch. Most children feel better right after surgery, although some may continue to have breathing symptoms.

In rare cases, if the arch is pressing down very hard on the airway, the child can have severe breathing difficulty that leads to death.

Complications:

Failure to thrive
Respiratory infections
Wearing away of the lining of the esophagus (esophageal erosion) and windpipe
Very rarely an abnormal connection between the esophagus and aorta (aortoesophageal fistula)

Calling your health care provider:

Call your health care provider if your infant has symptoms of double aortic arch.

Prevention:

There is no known way to prevent this condition.

References:

Park MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.

Bernstein D. Other congenital heart and vascular malformations. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Behrman: Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders; 2011:chap 426.

Townsend Jr. CM, Beauchamp RD, Evers BM, Mattox KL. Townsend: Sabiston Textbook of Surgery. 18th ed. Philadelphia, Pa: Saunders; 2008.