Congenital Anomalies: Vascular Rings

Key Points

• The diagnosis of a vascular ring requires a high index of suspicion from the treating clinicians.
• These children typically present with noisy breathing and varying degrees of respiratory distress. Older children may present with dysphagia.
• We believe that a computed tomographic (CT) scan with contrast is the single best diagnostic study to confirm the diagnosis and to define the anatomy.
• Patients who have a double aortic arch or a right aortic arch with left ligamentum are typically approached through a left thoracotomy incision. The vascular ring is divided between vascular clamps.
• We treat innominate artery compression syndrome through a right anterolateral thoracotomy with suspension of the innominate artery to the posterior sternum.
• Pulmonary artery sling is repaired through a median sternotomy approach with the use of cardiopulmonary bypass. Patients with pulmonary artery sling are evaluated for the frequently associated tracheal stenosis (>50%), which is a significant part of the pathophysiology.
• Optimal outcomes for these patients depend on a close collaboration among the cardiovascular/thoracic surgery, otolaryngology, anesthesia, and intensive care unit teams.

Congenital malformations of the aortic arch system cause symptomatic tracheal and esophageal compression. These congenital anomalies are commonly referred to as vascular rings. Depending on the degree of compression, they can cause a continuum of symptoms ranging from dramatic airway distress in the newborn to subtle swallowing disorders in the adolescent. The age at presentation and the severity of the symptoms depend mostly on the tightness of the ring. This, in turn, is related chiefly to the anatomy of the ring. The nomenclature for vascular rings has been standardized by the International Nomenclature and Database Committee for Pediatric Cardiac Surgery.[1] The four primary vascular ring classifications are double aortic arch, right aortic arch with left ligamentum, innominate artery compression, and pulmonary artery sling (Table 1). Each of the different vascular rings has anatomic variations, some of which are much more common than others. Double aortic arch and right aortic arch with left ligamentum form anatomically complete rings. Innominate artery compression syndrome and pulmonary artery sling are not true complete anatomic rings, but they have a clinical presentation, diagnostic evaluation, and operative strategy similar to those of complete vascular rings; hence, both categories are considered as a single group.

Almost all vascular rings require surgical intervention. The surgical approach varies depending on the precise anatomy of the ring. We have come to rely on multislice CT scanning with contrast as the single best diagnostic tool to identify the anatomy.[2] In most patients, vascular ring repair leads to almost complete resolution of symptoms after a period of time.[3] This chapter reviews the significant historical events in vascular ring surgery, the embryology and pathology of vascular rings, the clinical presentation and diagnostic evaluation, the surgical management, and the postoperative care and outcomes.