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Right Aortic Arch
Carol C. Wu, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Right aortic arch (RAA)
            • Synonyms

              • Right arch
            • Definitions

              • Congenital anomaly in which aortic arch courses to right of trachea; different types based on branching patterns
                • RAA with aberrant left subclavian artery (ALSA)
                  • 4 great vessel branches in following order: Left carotid, right carotid, right subclavian, aberrant left subclavian arteries
                  • ALSA has retroesophageal course
                  • ± diverticulum of Kommerell at origin of ALSA
                • RAA with mirror image branching
                  • Left innominate artery followed by right carotid & right subclavian arteries
                • RAA with isolation of left subclavian artery
                  • Left carotid artery followed by right carotid & right subclavian arteries
                  • Left subclavian artery, not connected to aorta, is connected to pulmonary artery by left ductus arteriosus
                • RAA with left descending aorta
                  • a.k.a. circumflex aorta or retroesophageal aortic segment
                  • Transverse arch courses posterior to esophagus then continues as left descending aorta
                  • May be variant of double arch with atretic left arch
                • RAA with aberrant brachiocephalic
                  • Right carotid artery followed by right subclavian artery & left innominate artery
                  • Left innominate artery has a retroesophageal course

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      • Angiographic Findings

                        • Fluoroscopic Findings

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Hellinger JC et al: Congenital thoracic vascular anomalies: evaluation with state-of-the-art MR imaging and MDCT. Radiol Clin North Am. 49(5):969-96, 2011
                                        2. Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010
                                        3. Türkvatan A et al: Congenital anomalies of the aortic arch: evaluation with the use of multidetector computed tomography. Korean J Radiol. 10(2):176-84, 2009
                                        4. Craatz S et al: Right-sided aortic arch and tetralogy of Fallot in humans--a morphological study of 10 cases. Cardiovasc Pathol. 12(4):226-32, 2003
                                        5. Singh B et al: Right aortic arch with isolated left brachiocephalic artery. Clin Anat. 14(1):47-51, 2001
                                        6. Grathwohl KW et al: Vascular rings of the thoracic aorta in adults. Am Surg. 65(11):1077-83, 1999
                                        7. Moes CA et al: Rare types of aortic arch anomalies. Pediatr Cardiol. 14(2):93-101, 1993
                                        8. Jaffe RB: Radiographic manifestations of congenital anomalies of the aortic arch. Radiol Clin North Am. 29(2):319-34, 1991
                                        9. Backer CL et al: Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. 97(5):725-31, 1989
                                        10. Gomes AS: MR imaging of congenital anomalies of the thoracic aorta and pulmonary arteries. Radiol Clin North Am. 27(6):1171-81, 1989
                                        11. Hastreiter AR et al: Right-sided aorta. I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta, and associated anomalies. Br Heart J. 28(6):722-39, 1966
                                        12. Felson B et al: The two types of right aortic arch. Radiology. 81:745-59, 1963
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Right aortic arch (RAA)
                                                  • Synonyms

                                                    • Right arch
                                                  • Definitions

                                                    • Congenital anomaly in which aortic arch courses to right of trachea; different types based on branching patterns
                                                      • RAA with aberrant left subclavian artery (ALSA)
                                                        • 4 great vessel branches in following order: Left carotid, right carotid, right subclavian, aberrant left subclavian arteries
                                                        • ALSA has retroesophageal course
                                                        • ± diverticulum of Kommerell at origin of ALSA
                                                      • RAA with mirror image branching
                                                        • Left innominate artery followed by right carotid & right subclavian arteries
                                                      • RAA with isolation of left subclavian artery
                                                        • Left carotid artery followed by right carotid & right subclavian arteries
                                                        • Left subclavian artery, not connected to aorta, is connected to pulmonary artery by left ductus arteriosus
                                                      • RAA with left descending aorta
                                                        • a.k.a. circumflex aorta or retroesophageal aortic segment
                                                        • Transverse arch courses posterior to esophagus then continues as left descending aorta
                                                        • May be variant of double arch with atretic left arch
                                                      • RAA with aberrant brachiocephalic
                                                        • Right carotid artery followed by right subclavian artery & left innominate artery
                                                        • Left innominate artery has a retroesophageal course

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            • Angiographic Findings

                                                              • Fluoroscopic Findings

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Hellinger JC et al: Congenital thoracic vascular anomalies: evaluation with state-of-the-art MR imaging and MDCT. Radiol Clin North Am. 49(5):969-96, 2011
                                                                              2. Kanne JP et al: Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 4(5):293-300, 2010
                                                                              3. Türkvatan A et al: Congenital anomalies of the aortic arch: evaluation with the use of multidetector computed tomography. Korean J Radiol. 10(2):176-84, 2009
                                                                              4. Craatz S et al: Right-sided aortic arch and tetralogy of Fallot in humans--a morphological study of 10 cases. Cardiovasc Pathol. 12(4):226-32, 2003
                                                                              5. Singh B et al: Right aortic arch with isolated left brachiocephalic artery. Clin Anat. 14(1):47-51, 2001
                                                                              6. Grathwohl KW et al: Vascular rings of the thoracic aorta in adults. Am Surg. 65(11):1077-83, 1999
                                                                              7. Moes CA et al: Rare types of aortic arch anomalies. Pediatr Cardiol. 14(2):93-101, 1993
                                                                              8. Jaffe RB: Radiographic manifestations of congenital anomalies of the aortic arch. Radiol Clin North Am. 29(2):319-34, 1991
                                                                              9. Backer CL et al: Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. 97(5):725-31, 1989
                                                                              10. Gomes AS: MR imaging of congenital anomalies of the thoracic aorta and pulmonary arteries. Radiol Clin North Am. 27(6):1171-81, 1989
                                                                              11. Hastreiter AR et al: Right-sided aorta. I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta, and associated anomalies. Br Heart J. 28(6):722-39, 1966
                                                                              12. Felson B et al: The two types of right aortic arch. Radiology. 81:745-59, 1963