Vascular Rings and Pulmonary Artery Sling

Abstract

Vascular rings are congenital vascular anomalies of the aortic arch system which compress the esophagus and trachea. The two major categories of vascular rings are double aortic arch and right aortic arch with left ligamentum. A diagnosis of a vascular ring requires a high index of suspicion by the clinician. Clues to this diagnosis are noisy breathing, a barky cough, recurrent upper respiratory tract infections, and dysphagia. Vascular rings are repaired through a thoracotomy with division of the ligamentum, division of the smaller of the two aortic arches for double aortic arch, and resection of a Kommerell diverticulum if present. Almost all patients have resolution of their symptoms over a period of 6 months to 1 year. Patients with a pulmonary artery sling frequently have associated tracheal stenosis from complete cartilaginous tracheal rings. Repair is performed through a median sternotomy with the use of cardiopulmonary bypass. Simultaneous repair of associated intracardiac lesions and slide tracheoplasty for congenital tracheal stenosis are performed. Care of these patients requires multidisciplinary cooperation between cardiothoracic surgery, otolaryngology, anesthesia, and critical care.