Key Points

• The diagnostic approach for vascular tracheoesophageal compressive syndromes usually involves some combination of bronchoscopy, echocardiography, and chest CT or MRI.
• Respiratory symptoms due to vascular compression range from subclinical to life threatening. A high index of suspicion for the possibility of vascular compression of the airway should be present for asthma that is “atypical” or unresponsive to medical management.
• More than 90% of all vascular rings are approached surgically via left posterolateral thoracotomy.
• Pulmonary artery sling is repaired by translocation of the left pulmonary artery to the main pulmonary artery via median sternotomy with cardiopulmonary bypass and concomitant repair of significant tracheal anomalies.
• Degree of underlying tracheal abnormality is the most important prognostic factor for patients with a pulmonary artery sling.
• Innominate artery compression of the trachea should be repaired by aortopexy for patients with respiratory symptoms and significant tracheal narrowing demonstrated during bronchoscopic evaluation.

Tracheoesophageal compression syndromes may result from a variety of congenital abnormalities of the great vessels. These vascular anomalies usually have no hemodynamic impact on affected individuals but come to clinical attention due to compression of the trachea and/or the esophagus. The vascular anomalies that give rise to tracheoesophageal compression syndromes include vascular rings arising from abnormalities of the aortic arch and its branches, pulmonary artery slings, and innominate artery compression of the trachea. A standardized nomenclature for these lesions has been produced that accurately categorizes the great majority of these lesions (Table Although potentially life threatening, in the absence of advanced underlying structural abnormalities of the trachea that may be associated with these conditions, surgical repair reliably results in definitive treatment for these lesions.