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

Pediatr Radiol. 2007 Jan;37(1):63-7. doi: 10.1007/s00247-006-0343-7. Epub 2006 Nov 3.


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.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Aorta, Thoracic / abnormalities*
  • Aortic Arch Syndromes / diagnosis*
  • Aortic Arch Syndromes / surgery
  • Contrast Media
  • Female
  • Gadolinium DTPA
  • Heart Defects, Congenital
  • Humans
  • Imaging, Three-Dimensional
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging / methods*
  • Male
  • Retrospective Studies
  • Subclavian Artery / abnormalities


  • Contrast Media
  • Gadolinium DTPA