Vascular Rings

Abstract

Vascular rings are congenital aortic arch anomalies that cause symptoms by compressing either the esophagus, the trachea, or both. The classic symptoms are noisy breathing (stridor), barky cough, and dysphagia. Diagnosis is best obtained by advanced medical imaging, either computed tomography (CT) scan or magnetic resonance imaging (MRI). This reveals the precise anatomy of the vascular ring and provides a road map for operative intervention. The most common approach is through a left thoracotomy with division of the ligamentum arteriosum and appropriate vascular structures. Some patients with a right aortic arch will require resection of a structure called a Kommerell diverticulum, which serves as the origin of the left subclavian artery. This requires transfer of the left subclavian artery to the left carotid artery. A rare vascular ring is the pulmonary artery sling, which requires reimplantation of the left pulmonary artery into the main pulmonary artery. Patients with pulmonary artery sling need to be carefully evaluated for stenosis of the trachea caused by complete congenital cartilage tracheal rings. Aberrant right subclavian artery is not a true vascular ring, but can cause in rare cases esophageal compression. This may require transfer of the right subclavian artery to the right carotid artery via a right thoracotomy. In nearly all instances, the surgical division of the vascular ring results in resolution of airway or esophageal symptoms.