Vascular rings of the thoracic aorta in adults

Vascular rings have been well documented to cause respiratory and gastrointestinal symptoms in infants and children. Few reports document symptomatic vascular rings in adults, and most have emphasized dysphagia as the predominant symptom. We present the case of a 36-year-old white male with a double aortic arch and progressive dyspnea on exertion. This led us to review previous reports of vascular rings in adults. Criteria for review consisted of anatomically complete vascular rings of the aortic arch in adults age 18 years or older. We identified 25 prior cases for review and included our recent patient. The most common vascular ring anomalies in our review of adults is double aortic arch (n = 12; 46%) followed by right aortic arch with aberrant left subclavian artery and ligamentum arteriosum (n = 8; 30%). Of 24 patients (66%), 16 were symptomatic. Reported symptoms involving the respiratory tract (n = 10 of 24; 42%) included dyspnea on exertion (n = 5), bronchitis (n = 2), recurrent pneumonia, stridor, and unspecified respiratory ailment (n = 1 each). Dysphagia was less common, occurring in eight patients (33%). Previously proposed mechanisms for respiratory tract symptoms include tracheomalacia, static or dynamic compression of the airways, intravascular volume infusion, and aspiration. We also propose exercise-induced dilatation of the aortic arch and age-dependent changes in thoracic compliance as potential mechanisms of dyspnea.