Double aortic arch is an abnormal formation of the aorta—the large artery that carries blood from the heart to the rest of the body. It is a congenital problem, which means that it is present at birth.

See also: Vascular ring

Double aortic arch is one of the most common types of vascular ring—a group of defects that affect the development of the baby’s aorta in the womb.

Normally, the aorta develops from one of several curved pieces of tissue (arches). In babies with this condition, it splits into several parts that go around the windpipe (trachea) and esophagus (tube that goes from the mouth to the stomach). The parts come together again in front of the spine.

As the baby grows in the womb, the body breaks down some of the arches, while others form into arteries. With vascular ring, some of the arches that should have changed into arteries or disappeared are still present at birth.

Babies with a double aortic arch have an aorta that is made up of two vessels instead of one. The two parts to the aorta have smaller arteries branching off of them. As a result, the two branches go around and press down on the windpipe and the tube that carries food from the mouth to the stomach (esophagus).

The right arch is bigger in most people with double aortic arch.

A double aortic arch may be seen in people with:

• A chromosome defect
• Tetralogy of Fallot
• Transposition of the great arteries
• Ventricular septal defect

Double aortic arch is very rare. Vascular rings make up just a few of all congenital heart problems. Of these, a little more than half are double aortic arch. The condition occurs just as often in males as in females.

Because symptoms of double aortic arch are often mild, the problem may not be discovered until the child is a few years old.

Pressure from the aortic arch on the windpipe (trachea) and esophagus can lead to trouble breathing and swallowing. How severe the symptoms are depends on how much the aortic arch is pressing down on these structures.

Breathing symptoms include:

• Bluish color to the skin (cyanosis)
• High-pitched sound during breathing (stridor)
• Noisy breathing
• Repeated pneumonias
• Wheezing

Digestive symptoms are rare, but may include:

• Choking
• Difficulty eating and swallowing
• Vomiting

A doctor will usually diagnose double aortic arch while the child is still an infant. However, the doctor may not notice any signs during a physical exam.

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)

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.

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 die.

• Abnormal connection between the esophagus and aorta (aortoesophageal fistula)
• Failure to thrive
• Respiratory infections
• Wearing away of the lining of the esophagus (esophageal erosion) and windpipe

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

There is no known way to prevent this condition.

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

Behrman RE, Kliegman RM, Jenson HB. Behrman: Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: Saunders; 2004.

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