Double aortic arch

  • Definition
    • 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.

  • Alternative Names
    • Aortic arch anomaly; Double arch; Congenital heart defect - double aortic arch; Birth defect heart - double aortic arch

  • Causes
    • Double aortic arch is a common form of a group of defects that affect the development of the aorta in the womb. These defects cause an abnormal formation called a vascular ring (a circle of blood vessels).

      Normally, the aorta develops from one of several curved pieces of tissue (arches). As babies develop in the womb, the arches split into several parts. The body breaks down some of the arches, while others form into arteries. A normally developed aorta is a single arch that leaves the heart and moves leftward.

      Double aortic arch

      In double aortic arch, some of the arches that should have disappeared are still present at birth in addition to the normal arch. 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 (esophagus) that carries food from the mouth to the stomach.

      A double aortic arch may occur in other congenital heart defects, including:

      Double aortic arch is very rare. Vascular rings make up a small percentage of all congenital heart problems. Of these, a little more than half are caused by double aortic arch. The condition occurs equally in males and females. It is often present in people with certain chromosome abnormalities.

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

      The double aortic arch may press on the trachea and esophagus, leading to trouble breathing and swallowing. The severity of the symptoms depends on how much the aortic arch is pressing on these structures.

      Vascular ring

      Breathing symptoms include:

      • High-pitched sound during breathing (stridor)
      • Noisy breathing
      • Repeated pneumonias
      • Wheezing

      Digestive symptoms may include:

      • Choking
      • Difficulty eating and swallowing
      • Vomiting
  • Exams and Tests
    • The symptoms may lead a health care provider 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 surgeon ties off the smaller branch and separates it from the larger branch. Then the surgeon closes the ends of the aorta with stitches. This relieves pressure on the esophagus and windpipe.

  • Outlook (Prognosis)
    • Most children feel better right after surgery, although some may continue to have breathing symptoms for some time after surgical repair. This is most often due to weakness of the trachea because of the pressure on it before surgical repair.

      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.

  • Possible Complications
    • Complications may include:

      • 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)
  • When to Contact a Medical Professional
    • Call your provider if your infant has symptoms of double aortic arch.

  • Prevention
    • There is no known way to prevent this condition.

  • References
    • Fraser CD Jr, Carberry KE. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL. Sabiston Textbook of Surgery. 19th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 59.

      Kliegman RM, Stanton BF, St Geme JW, Schor NF. Other congenital heart and vascular malformations. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 432.

      Park MK. Vascular ring. In: Park MK, ed. Park's Pediatric Cardiology for Practitioners. 6th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 16.

      Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 62.