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Double aortic arch
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.
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.
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:
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Tetralogy of Fallot
Tetralogy of Fallot
Tetralogy of Fallot is a type of congenital heart defect. Congenital means that it is present at birth.
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Truncus arteriosus
Truncus arteriosus
Truncus arteriosus is a rare type of heart disease that in which a single blood vessel (truncus arteriosus) comes out of the right and left ventricle...
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Transposition of the great arteries
Transposition of the great arteries
Transposition of the great vessels is a heart defect that occurs from birth (congenital). The 2 major vessels that carry blood away from the heart -...
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Ventricular septal defect
Ventricular septal defect
Ventricular septal defect is a hole in the wall that separates the right and left ventricles of the heart. Ventricular septal defect is one of the m...
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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.
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:
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Failure to thrive
Failure to thrive
Failure to thrive refers to children whose current weight or rate of weight gain is much lower than that of other children of similar age and gender....
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- 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.
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Congenital heart defect overview
Animation
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Vascular ring - illustration
Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.
Vascular ring
illustration
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Double aortic arch - illustration
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. The aorta splits into two vessels instead of one soon after it leaves the heart and surround the esophagus (swallowing tube) and trachea (breathing tube). The two branches eventually come back together.
Double aortic arch
illustration
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Vascular ring - illustration
Vascular ring is a term used to describe a number of abnormal formations of the aorta, the large artery that carries blood from the heart to the rest of the body, or of the pulmonary artery. The abnormal vessel(s) forms a ring, which encircles and may press down on the windpipe (trachea) or the esophagus. The additional pressure on the windpipe (trachea) and esophagus can lead to breathing and swallowing problems.
Vascular ring
illustration
-
Double aortic arch - illustration
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. The aorta splits into two vessels instead of one soon after it leaves the heart and surround the esophagus (swallowing tube) and trachea (breathing tube). The two branches eventually come back together.
Double aortic arch
illustration
Review Date: 3/15/2016
Reviewed By: Scott I. Aydin, MD, Assistant Professor of Pediatrics, Albert Einstein College of Medicine, Division of Pediatric Cardiology and Critical Care Medicine, The Children'’s Hospital at Montefiore, Bronx, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
