Double aortic arch

Double Aortic Arch is a rare congenital cardiovascular malformation where two aortic arches form a vascular ring around the trachea and esophagus. This condition is a type of vascular ring anomaly, which can lead to symptoms related to compression of these structures, such as breathing difficulties, swallowing problems, and persistent cough. The double aortic arch is the most common form of a complete vascular ring, making early diagnosis and treatment crucial for affected individuals.

Etiology

The development of a double aortic arch occurs during the embryonic stage of development. Normally, the aortic arch develops from one of several embryonic pharyngeal arch arteries. In the case of double aortic arch, both the right and left fourth pharyngeal arch arteries persist, instead of one regressing, leading to the formation of two aortic arches. This condition is thought to be sporadic, with no clear genetic predisposition identified, although it can occasionally be seen in association with other congenital anomalies.

Pathophysiology

In a double aortic arch, both arches encircle the trachea and esophagus, forming a vascular ring. This ring can compress these structures to varying degrees, depending on the size and position of the arches. The right aortic arch is usually dominant in most cases, but both arches can be of equal size. The degree of symptoms is related to the severity of the compression.

Clinical Presentation

Symptoms of double aortic arch can present early in life, often within the first few months after birth. Common symptoms include:

• Stridor
• Wheezing
• Recurrent respiratory infections
• Dysphagia (difficulty swallowing)
• Feeding difficulties in infants

In severe cases, significant tracheal compression can lead to life-threatening respiratory distress.

Diagnosis

Diagnosis of double aortic arch typically involves imaging studies to visualize the vascular ring and its effect on surrounding structures. Key diagnostic tools include:

• Echocardiography: Can suggest the presence of a vascular ring.
• Computed Tomography (CT) Scan or Magnetic Resonance Imaging (MRI): These imaging modalities provide detailed images of the vascular anatomy, confirming the diagnosis and aiding in surgical planning.
• Barium Swallow Study: Helps in assessing the degree of esophageal compression.

Treatment

The definitive treatment for double aortic arch is surgical intervention to relieve the compression of the trachea and esophagus. The surgical approach typically involves division of the smaller arch, which is usually the left arch, to open the vascular ring. Postoperative outcomes are generally excellent, with most children experiencing significant relief from symptoms.

Prognosis

With timely and appropriate surgical treatment, the prognosis for individuals with double aortic arch is generally very good. Most children lead normal, healthy lives post-surgery, although long-term follow-up is recommended to monitor for any potential complications, such as recurrent tracheal compression or the development of aortic aneurysms at the site of the surgical repair.

Conclusion

Double aortic arch is a rare but treatable cause of tracheoesophageal compression in infants and young children. Early recognition and surgical intervention are crucial to prevent complications and ensure a good quality of life for affected individuals.