Common aortic arch anomalies: diagnosis and management

Vascular rings are a rare form of congenital malformation that completely encircle the trachea and esophagus with vascular structures. The two most common forms are double aortic arch and right aortic arch with an aberrant left subclavian artery and a left-sided ligamentum arteriosum. Patients may present with symptoms of airway or esophageal impingement, including "noisy breathing," stridor, respiratory distress, difficulty feeding, or dysphagia. Typically, the more severe the airway or esophageal compression, the earlier the age at presentation. Diagnosis was traditionally made by posterior compression of the esophagus demonstrated by barium swallow; in the current era, CT angiography or magnetic resonance angiography is used. Although helpful to delineate intracardiac anatomy and associated structural heart defects, echocardiography is less useful for imaging vascular structures when atretic segments comprise part of the vascular ring. Management includes surgical division of the structures contributing to the vascular ring. Some centers also perform surgical remodeling of Kommerell's diverticulum because this structure may contribute to continuing tracheal or esophageal compression even after the ring has been released. Patients with associated tracheomalacia may continue to have symptoms that can last for months, but ultimately resolve with time. Long-term results are generally excellent with minimal morbidity and mortality.