Aortic wheeze: intermittent tracheal obstruction caused by a rare aortic arch anomaly

Cardiac and aortic arch anomalies are a well-known cause of tracheal and esophageal compression producing stridor and dysphagia. We present a rarely encountered aortic arch anomaly in a 12-month-old infant who suffered from intermittent pulmonary wheezing. A discussion of the subsequent evaluation including an esophageal contrast study, computed tomography, angiocardiography, and bronchoscopy, will emphasize the diagnostic importance of these examinations. Preoperatively, a variant of Celoria type B aortic arch interruption was established by cardiac catheterization. The ascending aorta solely supplied both carotid arteries. Both subclavian arteries arose from the descending aorta, and all flow to the descending aorta was supplied via bilateral markedly enlarged vertebral arteries as a bilateral subclavian steal. Three areas of tracheal compression were noted at bronchoscopy. The patient's anomalous circulation was improved by the placement of a Gore-Tex graft connecting the ascending and descending aorta. A right-sided arterial ligament was divided in order to further relieve tracheal compression.