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Double Aortic Arch
Carlos S. Restrepo, MD; 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 and left 4th embryonic aortic arches
                • Complete vascular ring encircles trachea and esophagus
              • Most common symptomatic vascular ring (55%)
              • May occur with or without other congenital abnormalities
              • Often isolated anomaly 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. Young AA et al: Prenatal detection, comorbidities, and management of vascular rings. Am J Cardiol. 123(10):1703-8, 2019
                                            2. Priya S et al: Congenital anomalies of the aortic arch. Cardiovasc Diagn Ther. 8(Suppl 1):S26-44, 2018
                                            3. Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017
                                            4. Backer CL et al: Vascular rings. Semin Pediatr Surg. 25(3):165-75, 2016
                                            5. Smith BM et al: Rings and slings revisited. Magn Reson Imaging Clin N Am. 23(1):127-35, 2015
                                            6. Etesami M et al: Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 5(4):507-21, 2014
                                            7. Murillo H et al: Imaging of the aorta: embryology and anatomy. Semin Ultrasound CT MR. 33(3):169-90, 2012
                                            8. Restrepo CS et al: Multidetector computed tomography of congenital anomalies of the thoracic aorta. Semin Ultrasound CT MR. 33(3):191-206, 2012
                                            9. Cerillo AG et al: Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 99(2):191-4, 2005
                                            10. 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
                                            11. Greil GF et al: Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique. Pediatr Radiol. 2005
                                            12. Subramanyan R et al: Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr. 40(10):951-7, 2003
                                            13. 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
                                            14. 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
                                            15. Skinner LJ et al: Complete vascular ring detected by barium esophagography. Ear Nose Throat J. 81(8):554-5, 2002
                                            16. McElhinney DB et al: Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol. 37(8):2114-9, 2001
                                            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 and left 4th embryonic aortic arches
                                                          • Complete vascular ring encircles trachea and esophagus
                                                        • Most common symptomatic vascular ring (55%)
                                                        • May occur with or without other congenital abnormalities
                                                        • Often isolated anomaly 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. Young AA et al: Prenatal detection, comorbidities, and management of vascular rings. Am J Cardiol. 123(10):1703-8, 2019
                                                                                      2. Priya S et al: Congenital anomalies of the aortic arch. Cardiovasc Diagn Ther. 8(Suppl 1):S26-44, 2018
                                                                                      3. Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017
                                                                                      4. Backer CL et al: Vascular rings. Semin Pediatr Surg. 25(3):165-75, 2016
                                                                                      5. Smith BM et al: Rings and slings revisited. Magn Reson Imaging Clin N Am. 23(1):127-35, 2015
                                                                                      6. Etesami M et al: Computed tomography in the evaluation of vascular rings and slings. Insights Imaging. 5(4):507-21, 2014
                                                                                      7. Murillo H et al: Imaging of the aorta: embryology and anatomy. Semin Ultrasound CT MR. 33(3):169-90, 2012
                                                                                      8. Restrepo CS et al: Multidetector computed tomography of congenital anomalies of the thoracic aorta. Semin Ultrasound CT MR. 33(3):191-206, 2012
                                                                                      9. Cerillo AG et al: Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 99(2):191-4, 2005
                                                                                      10. 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
                                                                                      11. Greil GF et al: Diagnosis of vascular rings and slings using an interleaved 3D double-slab FISP MR angiography technique. Pediatr Radiol. 2005
                                                                                      12. Subramanyan R et al: Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr. 40(10):951-7, 2003
                                                                                      13. 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
                                                                                      14. 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
                                                                                      15. Skinner LJ et al: Complete vascular ring detected by barium esophagography. Ear Nose Throat J. 81(8):554-5, 2002
                                                                                      16. McElhinney DB et al: Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol. 37(8):2114-9, 2001