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Key Concepts

  • Epidemiology

    • Vascular rings and pulmonary artery sling (PAS) represent 1 to 3 percent of all congenital cardiac malformations. The most frequent type of vascular ring is aberrant left subclavian artery, whereas the most rare condition is the PAS.

  • Morphology

    • Of the over 100 variations in morphology that have been described, 6 are most commonly observed: (1) anomalous left subclavian artery with left aortic arch, (2) double aortic arch (DAA), (3) right aortic arch with left ligamentum, (4) right aortic arch with aberrant left subclavian artery, (5) tracheal compression by the innominate artery, and (6) PAS. Cardiac malformations (tetralogy of Fallot, ventricular septal defect, atrial septal defect, and aortic coarctation) are seen in 15 to 20 percent of cases.

  • Pathophysiology

    • Structural obstruction of the aerodigestive tract

  • Clinical features

    • Most vascular rings are symptomatic. Compression by the complete or partial ring of the esophagus, trachea, or both dictates the mode of presentation. Patients with an aberrant left subclavian artery are often asymptomatic, whereas pulmonary artery sling and complete rings are almost uniformly clinically significant.

  • Diagnosis

    • Chest x-ray is rarely diagnostic. Barium esophagography is usually able to point to the presence of a vascular ring with reasonable specificity. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful in defining the anatomy and are currently more routinely utilized. Bronchoscopy is important in the diagnosis of both innominate artery compression and PAS and in assessing the degree of tracheobronchomalacia.

  • Treatment

    • Treatment is indicated in all symptomatic patients. With the exception of DAA with dominant left component, almost all cases (left aortic arch with right ligamentum, PAS, and innominate artery compression) are approached through a left thoracotomy with division of the vascular ring.

  • Outcomes

    • For the vast majority of vascular rings, morbidity and mortality are the same as seen in any simple thoracic procedure involving a thoracotomy. Mortality is much higher for patients with PAS. The degree of tracheobronchomalacia has a significant impact on long-term prognosis.

Introduction and Definitions

Vascular rings are congenital abnormalities of the aortic arch system that cause tracheal and esophageal compression. Patients with vascular rings have varying degrees of symptoms that range from subtle swallowing difficulties in the adolescent to severe respiratory distress in the neonate. The symptoms depend on the tightness of the vascular ring as it compresses the esophagus and/or trachea. The phrase “pulmonary artery sling (PAS)” refers to a particular anomaly where the left pulmonary artery (PA) originates from the right PA and compresses the distal trachea and right bronchus on its route to the left lung. The Congenital Heart Surgery Nomenclature and Database Project Committee has standardized the classification of vascular rings to include complete rings [double aortic arch (DAA), right aortic arch with left ligamentum] and incomplete rings [pulmonary artery sling (PAS), innominate artery compression].1 Each of these vascular rings can have different anatomic variations. Nearly all patients with complete vascular rings and PAS will eventually require ...

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