What are the other Names for this Condition? (Also known as/Synonyms)

• Aortic Arch Anomaly
• DAA (Double Aortic Arch)
• Double-Sided Aortic Arch

What is Double Aortic Arch? (Definition/Background Information)

• Double Aortic Arch (DAA) is a type of birth defect that involves the development of the aorta.  The defects lead to an abnormal formation of a circle of blood vessels known as the vascular ring
• During normal development of the aorta, primitive arches of aorta develop. As the aorta develops, portions of some parts disappear, whereas other parts develop into the aortic arch
• When abnormal embryological development results in the persistence of portions of aortic arch that should have disappeared, it results in a Double Aortic Arch. Normally, there should be a single aortic arch; the presence of a DAA is abnormal. This abnormal second aortic arch can wrap around and compress the windpipe (trachea) and/or the foodpipe (esophagus)
• The risk factors for Double Aortic Arch are currently unknown. The cause of this heart abnormality is also poorly understood
• Double Aortic Arch can remain silent for many years without the appearance of any symptoms.  In case of symptoms, it may include difficulty breathing, lung infections, and vomiting. In case of complications, it may result in esophageal erosion
• The treatment of Double Aortic Arch usually involves surgery. The prognosis is good, if the condition is managed properly

Who gets Double Aortic Arch? (Age and Sex Distribution)

• Double Aortic Arch is a very rare congenital (develops prior to birth) condition
• The condition occurs in both males and females.
• All races and ethnicities are affected and no preference is seen

What are the Risk Factors for Double Aortic Arch? (Predisposing Factors)

• Presently, the risk factors for the development of a Double Aortic Arch are not well understood

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Double Aortic Arch? (Etiology)

• Double Aortic Arch is a congenital birth defect; individuals that exhibit this defect are born with the condition
• The reason why the aorta undergoes abnormal development is currently unknown

What are the Signs and Symptoms of Double Aortic Arch?

A child born with Double Aortic Arch can live for several years without any visible symptoms. The signs and symptoms that begin to manifest early include difficulty breathing and swallowing. The other indications may include:

• Lung infections
• Poor feeding
• Vomiting, choking
• High-pitched breathing (stridor)

How is Double Aortic Arch Diagnosed?

There are several methods to diagnose a Double Aortic Arch and these include:

• Physical examination and complete family history evaluation
• X-ray of the chest
• Ultrasound of the heart
• CT scan or MRI scan of the heart
• Angiography involving the aortic arches
• Barium swallow study (the swallowed barium substance outlines the esophagus, which is then imaged using an x-ray)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are possible Complications of Double Aortic Arch?

The complications due to Double Aortic Arch may include the following:

• Children with DAA may experience difficultly in gaining and maintaining a healthy weight
• It may lead to wearing of the lining of the esophagus (esophageal erosion) and the lining of the windpipe
• In rare cases, the esophagus and the aorta may become connected (aortoesophageal fistula)

How is Double Aortic Arch Treated?

The type of treatment for Double Aortic Arch and the age at which treatment occurs varies on a case by case basis.

• If symptoms are mild, monitoring the Double Aortic Arch throughout one’s life is a sufficient plan
• Surgery can be performed to relieve the pinching of the windpipe and esophagus by the aorta. The smaller branch is disconnected from the larger branch; the ends of the aorta are then sealed with stitches

How can Double Aortic Arch be Prevented?

• Currently, there are no known methods of preventing the formation of a Double Aortic Arch
• To minimize the risk of deleterious effects on the fetus, expectant mothers should receive appropriate prenatal care and avoid substances that can lead to birth defects

What is the Prognosis of Double Aortic Arch? (Outcomes/Resolutions)

• If Double Aortic Arch is managed adequately and maintained under control, the prognosis for this heart defect is typically good. DAA does not present a great risk to the individual
• However, in certain rare circumstances, it may prove fatal if an obstruction of the windpipe occurs, thus preventing breathing

Additional and Relevant Useful Information for Double Aortic Arch:

Please visit our Heart & Vascular Health Center for more physician-approved health information:

http://www.dovemed.com/healthy-living/heart-center/