Aortic arch anomaly; Double arch; Congenital heart defect - double aortic arch; Birth defect heart - double aortic arch
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.
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.
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.
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.
Breathing symptoms include:
Digestive symptoms may include:
The symptoms may lead a health care provider to suspect double aortic arch. Other tests will then be needed to confirm the diagnosis.
The following tests can help diagnose double aortic arch:
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.
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.
Complications may include:
Call your provider if your infant has symptoms of double aortic arch.
There is no known way to prevent this condition.
Bryant R, Yoo S-J. Vascular rings, pulmonary arterial sling, and related conditions. In: Wernovsky G, Anderson RH, Kumar K, Mussatto K, et al, eds. Anderson's Pediatric Cardiology. 4th ed. Philadelphia, PA: Elsevier; 2020:chap 47.
Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, Beauchamp RD, Evers BM, Mattox KL, eds. Sabiston Textbook of Surgery. 20th ed. Philadelphia, PA: Elsevier; 2017:chap 58.
Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Other congenital heart and vascular malformations. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 459.
Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.BACK TO TOP
Review Date: 1/27/2020
Reviewed By: Michael A. Chen, MD, PhD, Associate Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- 2022 A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.