What is a Double Aortic Arch?

J.M. Willhite

A double aortic arch is a rare congenital cardiovascular malformation that may present symptoms alongside several other forms of congenital abnormalities. Impairing an infant’s digestive and respiratory functions, a double aortic arch is often mild in its presentation, allowing it to remain undiagnosed until the child is older. Treatment for this condition often necessitates the surgical separation of the arches to alleviate symptoms and restore proper respiratory function. If left untreated, a double aortic arch does carry a significant risk for complications, including an increased susceptibility for infection and esophageal deterioration.

A diagram of the aorta, showing a normal aortic arch.
A diagram of the aorta, showing a normal aortic arch.

While in utero, a fetusaorta normally forms as a single arch that curves leftward, leading away from the heart muscle. In the presence of a double aortic arch, an excess arch remains in place, compromising existing arterial function. The two arches possess their own smaller arterial passages that loop around and place unnecessary pressure on the esophagus and trachea. It is not uncommon for a double aortic arch to occur along with other congenital heart malformations or conditions, such as a ventricular septal defect or chromosomal mosaicism.

Individuals with a double aortic arch may experience noisy respiration and choking.
Individuals with a double aortic arch may experience noisy respiration and choking.

Due to the subdued nature of initial symptom presentation, a double aortic arch may remain undiagnosed for several years. Once symptoms are present, a variety of diagnostic tests may be used following an initial physical examination to confirm the presence of this congenital abnormality. Generally, imaging tests, including X-ray and magnetic resonance imaging (MRI), may be performed to evaluate the condition and functionality of the heart muscle. Additional imaging tests may be conducted to assess the condition of the respiratory tract, including the esophagus and trachea.

Those with a double aortic arch may experience a variety of signs and symptoms that progressively worsen with increased pressure. Though the initial presentation of the congenital defect may be mild, the effects of esophageal and tracheal pressure may progressively compromise respiratory function. Individuals with this condition may exhibit noisy or raspy respiration and have issues with choking and vomiting. Some symptomatic individuals may experience the development of chronic bouts of pneumonia as a result of persistent airway restriction. If left untreated, continued pressure on the esophagus can lead to an erosion of esophageal and tracheal tissues and chronic respiratory infections.

The surgical separation of the double aortic arch is the only viable treatment option to alleviate symptoms and eliminate esophageal and tracheal pressures. Performed under general anesthesia, the surgery involves separating the secondary branch from the primary one and using sutures to close the aortic openings that remain at either end. As with any invasive procedure, surgery to correct this congenital abnormality does carry risk for significant complications, including infection, excessive bleeding, and blood clots. Individuals who undergo surgery generally experience an immediate alleviation of symptoms and improvement in their respiratory function.

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