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Double Aortic Arch
Diane C. Strollo, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Congenital aortic arch anomaly
                • Persistent right & left 4th embryonic aortic arches
                • Complete vascular ring encases trachea & esophagus
              • Most common symptomatic vascular ring (55%)
              • Typically occurs with other congenital abnormalities
              • May be isolated anomaly, especially in adults

            IMAGING

            • General Features

              • Radiographic Findings

                • Fluoroscopic Findings

                  • CT Findings

                    • MR Findings

                      • Echocardiographic Findings

                        • Angiographic Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                • Gross Pathologic & Surgical Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Cerillo AG et al: Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 99(2):191-4, 2005
                                            2. Chan MS et al: Angiography and dynamic airway evaluation with MDCT in the diagnosis of double aortic arch associated with tracheomalacia. AJR Am J Roentgenol. 185(5):1248-51, 2005
                                            3. Greil GF et al: Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique. Pediatr Radiol. 2005
                                            4. Schlesinger AE et al: Incomplete double aortic arch with atresia of the distal left arch: distinctive imaging appearance. AJR Am J Roentgenol. 184(5):1634-9, 2005
                                            5. Backer CL et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 234-50, 2003
                                            6. Subramanyan R et al: Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr. 40(10):951-7, 2003
                                            7. Yilmaz M et al: Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management. Heart Surg Forum. 6(3):149-52, 2003
                                            8. Fleck RJ et al: Imaging findings in pediatric patients with persistent airway symptoms after surgery for double aortic arch. AJR Am J Roentgenol. 178(5):1275-9, 2002
                                            9. Park SC et al: Pediatric Cardiology. Vol 2. 2nd ed. London: Churchill Livingstone. 1559-75, 2002
                                            10. Skinner LJ et al: Complete vascular ring detected by barium esophagography. Ear Nose Throat J. 81(8):554-5, 2002
                                            11. Brockes C et al: Double aortic arch: diagnosis missed for 29 years. Vasa. 29(1):77-9, 2000
                                            12. Gustafson LM et al: Spiral CT versus MRI in neonatal airway evaluation. Int J Pediatr Otorhinolaryngol. 52(2):197-201, 2000
                                            13. McMahon CJ et al: Double aortic arch in D-transposition of the great arteries: confirmation of dominant arch by magnetic resonance imaging. Tex Heart Inst J. 27(4):398-400, 2000
                                            14. Krinsky GA et al: Thoracic aorta: comparison of single-dose breath-hold and double-dose non-breath-hold gadolinium-enhanced three-dimensional MR angiography. AJR Am J Roentgenol. 173(1):145-50, 1999
                                            15. Beghetti M et al: Double aortic arch. J Pediatr. 133(6):799, 1998
                                            16. Donnelly LF et al: The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol. 168(1):59-62, 1997
                                            17. Fattori R et al: Intramural posttraumatic hematoma of the ascending aorta in a patient with a double aortic arch. Eur Radiol. 7(1):51-3, 1997
                                            18. Hopkins KL et al: Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology. 200(3):811-5, 1996
                                            19. Murdison KA: Ultrasonic Imaging of Vascular Rings and Other Anomalies Causing Tracheobronchial Compression. Echocardiography. 13(3):337-356, 1996
                                            20. Othersen HB Jr et al: Aortoesophageal fistula and double aortic arch: two important points in management. J Pediatr Surg. 31(4):594-5, 1996
                                            21. Ito K et al: A case of the incomplete double aortic arch diagnosed in adulthood by MR imaging. Radiat Med. 13(5):263-7, 1995
                                            22. Katz M et al: Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr. 19(4):564-8, 1995
                                            23. Simoneaux SF et al: MR imaging of the pediatric airway. Radiographics. 15(2):287-98; discussion 298-9, 1995
                                            24. Tuma S et al: Double aortic arch in d-transposition of the great arteries complicated by tracheobronchomalacia. Cardiovasc Intervent Radiol. 18(2):115-7, 1995
                                            25. van Son JA et al: Demonstration of vascular ring anatomy with ultrafast computed tomography. Thorac Cardiovasc Surg. 43(2):120-1, 1995
                                            26. van Son JA et al: Imaging strategies for vascular rings. Ann Thorac Surg. 57(3):604-10, 1994
                                            27. Kramer LA et al: Rare case of double aortic arch with hypoplastic right dorsal segment and associated tetralogy of Fallot: MR findings. Magn Reson Imaging. 11(8):1217-21, 1993
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Definitions

                                                        • Congenital aortic arch anomaly
                                                          • Persistent right & left 4th embryonic aortic arches
                                                          • Complete vascular ring encases trachea & esophagus
                                                        • Most common symptomatic vascular ring (55%)
                                                        • Typically occurs with other congenital abnormalities
                                                        • May be isolated anomaly, especially in adults

                                                      IMAGING

                                                      • General Features

                                                        • Radiographic Findings

                                                          • Fluoroscopic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Echocardiographic Findings

                                                                  • Angiographic Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Cerillo AG et al: Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 99(2):191-4, 2005
                                                                                      2. Chan MS et al: Angiography and dynamic airway evaluation with MDCT in the diagnosis of double aortic arch associated with tracheomalacia. AJR Am J Roentgenol. 185(5):1248-51, 2005
                                                                                      3. Greil GF et al: Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique. Pediatr Radiol. 2005
                                                                                      4. Schlesinger AE et al: Incomplete double aortic arch with atresia of the distal left arch: distinctive imaging appearance. AJR Am J Roentgenol. 184(5):1634-9, 2005
                                                                                      5. Backer CL et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 234-50, 2003
                                                                                      6. Subramanyan R et al: Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr. 40(10):951-7, 2003
                                                                                      7. Yilmaz M et al: Vascular anomalies causing tracheoesophageal compression: a 20-year experience in diagnosis and management. Heart Surg Forum. 6(3):149-52, 2003
                                                                                      8. Fleck RJ et al: Imaging findings in pediatric patients with persistent airway symptoms after surgery for double aortic arch. AJR Am J Roentgenol. 178(5):1275-9, 2002
                                                                                      9. Park SC et al: Pediatric Cardiology. Vol 2. 2nd ed. London: Churchill Livingstone. 1559-75, 2002
                                                                                      10. Skinner LJ et al: Complete vascular ring detected by barium esophagography. Ear Nose Throat J. 81(8):554-5, 2002
                                                                                      11. Brockes C et al: Double aortic arch: diagnosis missed for 29 years. Vasa. 29(1):77-9, 2000
                                                                                      12. Gustafson LM et al: Spiral CT versus MRI in neonatal airway evaluation. Int J Pediatr Otorhinolaryngol. 52(2):197-201, 2000
                                                                                      13. McMahon CJ et al: Double aortic arch in D-transposition of the great arteries: confirmation of dominant arch by magnetic resonance imaging. Tex Heart Inst J. 27(4):398-400, 2000
                                                                                      14. Krinsky GA et al: Thoracic aorta: comparison of single-dose breath-hold and double-dose non-breath-hold gadolinium-enhanced three-dimensional MR angiography. AJR Am J Roentgenol. 173(1):145-50, 1999
                                                                                      15. Beghetti M et al: Double aortic arch. J Pediatr. 133(6):799, 1998
                                                                                      16. Donnelly LF et al: The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol. 168(1):59-62, 1997
                                                                                      17. Fattori R et al: Intramural posttraumatic hematoma of the ascending aorta in a patient with a double aortic arch. Eur Radiol. 7(1):51-3, 1997
                                                                                      18. Hopkins KL et al: Pediatric great vessel anomalies: initial clinical experience with spiral CT angiography. Radiology. 200(3):811-5, 1996
                                                                                      19. Murdison KA: Ultrasonic Imaging of Vascular Rings and Other Anomalies Causing Tracheobronchial Compression. Echocardiography. 13(3):337-356, 1996
                                                                                      20. Othersen HB Jr et al: Aortoesophageal fistula and double aortic arch: two important points in management. J Pediatr Surg. 31(4):594-5, 1996
                                                                                      21. Ito K et al: A case of the incomplete double aortic arch diagnosed in adulthood by MR imaging. Radiat Med. 13(5):263-7, 1995
                                                                                      22. Katz M et al: Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr. 19(4):564-8, 1995
                                                                                      23. Simoneaux SF et al: MR imaging of the pediatric airway. Radiographics. 15(2):287-98; discussion 298-9, 1995
                                                                                      24. Tuma S et al: Double aortic arch in d-transposition of the great arteries complicated by tracheobronchomalacia. Cardiovasc Intervent Radiol. 18(2):115-7, 1995
                                                                                      25. van Son JA et al: Demonstration of vascular ring anatomy with ultrafast computed tomography. Thorac Cardiovasc Surg. 43(2):120-1, 1995
                                                                                      26. van Son JA et al: Imaging strategies for vascular rings. Ann Thorac Surg. 57(3):604-10, 1994
                                                                                      27. Kramer LA et al: Rare case of double aortic arch with hypoplastic right dorsal segment and associated tetralogy of Fallot: MR findings. Magn Reson Imaging. 11(8):1217-21, 1993