Markedly hypoplastic circumflex retroesophageal right aortic arch: MR imaging and surgical implications

Background Circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment is a rare anomaly of the aortic arch. Adequate surgical management relies on precise diagnosis, which might not be feasible with echocardiography. Objective To demonstrate the utility of MR imaging in establishing the diagnosis and to describe potential pitfalls in making the diagnosis. Materials and methods Three patients with a circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment were retrospectively evaluated. All patients underwent evaluation by echocardiography and MR imaging. The MR imaging consisted of 3-D MR angiography in two patients and fast gradient recalled echo with cardiac-triggered segmented acquisition in one patient. Surgical confirmation was obtained on all three patients. Results The arch anatomy was accurately depicted in all three patients by MR imaging and in none of the patients by echocardiography. Conclusion MR imaging is extremely useful in establishing the diagnosis of markedly hypoplastic retroesophageal circumflex right aortic arch and thus helps in surgical planning.