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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.


Review Date: 2/7/2012
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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