Sharmila Udupa’s research while affiliated with University of Ottawa and other places

A right aortic arch with a retroesophageal brachiocephalic artery is a very rare type of vascular ring. We present a case report along with a review of published cases to date. Twelve publications from 1968-2014 describe a retroesophageal brachiocephalic artery in a total of 13 patients. The mean age at diagnosis was 8.7 ± 16 years. Four of the 13 patients were boys. Nearly half of the patients were symptomatic, with dysphagia or respiratory difficulties. Ten patients (77%) had associated congenital heart defects. Of the 13 patients with retroesophageal brachiocephalic artery, 12 had a right aortic arch, and only 1 had a left aortic arch associated with complex congenital heart disease. Investigations used in the diagnosis of the vascular ring include angiography, esophagography, echocardiography, and computed tomography. Only 2 patients had the diagnosis confirmed with magnetic resonance imaging (MRI), but this was in the setting of complex congenital heart disease. In conclusion, a right aortic arch with a retroesophageal left brachiocephalic artery is an extremely rare form of vascular ring and is often seen in association with other forms of congenital heart disease. Cardiac MRI can be used as a primary diagnostic modality for both the assessment of the vascular ring anatomy and the hemodynamics of associated cardiac malformations in the preoperative setting. Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.