Double aortic arch is a rare congenital cardiovascular malformation that can compromise a child’s digestive and respiratory functions. It often remains undiagnosed until the child is older. Treatment requires surgical separation of the arches to relieve symptoms and restore proper respiratory function.
A double aortic arch is a rare congenital cardiovascular malformation that can present with symptoms along with several other forms of congenital anomalies. Compromising a child’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 requires surgical separation of the arches to relieve symptoms and restore proper respiratory function. Left untreated, a double aortic arch carries a significant risk of complications, including increased susceptibility to infection and deterioration of the esophagus.
While in utero, a fetus’s aorta normally forms as a single arc that curves to the left, 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 have their own smaller arterial passages that wrap around and put 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.
Due to the blunt nature of the initial presentation of symptoms, a double aortic arch can go undiagnosed for several years. Once symptoms are present, a variety of diagnostic tests may be used after an initial physical exam to confirm the presence of this congenital abnormality. Usually, imaging tests, including X-rays and magnetic resonance imaging (MRI), may be done to evaluate the condition and function of the heart muscle. Additional imaging tests may be conducted to evaluate 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 get progressively worse as pressure increases. Although the initial presentation of the birth defect may be mild, the effects of esophageal and tracheal pressure can progressively compromise respiratory function. Individuals with this condition may experience noisy or hoarse breathing and have problems with choking and vomiting. Some symptomatic individuals may experience the development of chronic bouts of pneumonia due to persistent airway narrowing. If left untreated, continued pressure on the esophagus can lead to erosion of the esophageal and tracheal tissues and chronic respiratory infections.
Surgical separation of the double aortic arch is the only viable treatment option to relieve symptoms and eliminate esophageal and tracheal pressures. Performed under general anesthesia, the surgery involves separating the secondary branch from the primary branch and using sutures to close the aortic openings that remain at both ends. As with any invasive procedure, surgery to correct this congenital abnormality carries the risk of significant complications, including infections, excessive bleeding, and blood clots. Individuals who undergo surgery generally experience immediate relief of symptoms and improvement in their respiratory function.
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