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Double Aortic Arch
Mantosh S. Rattan, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Double aortic arch (DAA)
          • Definitions

            • Congenital aortic arch anomaly related to persistence of both left & right 4th aortic arches

          IMAGING

          • General Features

            • Radiographic Findings

              • Fluoroscopic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic 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. Trobo Marina D et al: Neonatal magnetic resonance imaging in double aortic arch diagnosed prenatally by ultrasound. J Obstet Gynaecol. 36(4):526-8, 2016
                                            2. Gould SW et al: Useful signs for the assessment of vascular rings on cross-sectional imaging. Pediatr Radiol. 45(13):2004-16, 2015
                                            3. Iwaki R et al: Follow-up of persistent tracheal stenosis after surgery for a double aortic arch. World J Pediatr Congenit Heart Surg. 6(3):458-61, 2015
                                            4. Trobo D et al: Prenatal sonographic features of a double aortic arch: literature review and perinatal management. J Ultrasound Med. 34(11):1921-7, 2015
                                            5. Kir M et al: Vascular rings: presentation, imaging strategies, treatment, and outcome. Pediatr Cardiol. 33(4):607-17, 2012
                                            6. Lee EY et al: MDCT evaluation of the prevalence of tracheomalacia in children with mediastinal aortic vascular anomalies. J Thorac Imaging. 23(4):258-65, 2008
                                            7. Alsenaidi K et al: Management and outcomes of double aortic arch in 81 patients. Pediatrics. 118(5):e1336-41, 2006
                                            8. Malik TH et al: The role of magnetic resonance imaging in the assessment of suspected extrinsic tracheobronchial compression due to vascular anomalies. Arch Dis Child. 91(1):52-5, 2006
                                            9. van Woerkum F et al: Aortoesophageal fistula due to double aortic arch and prolonged nasogastric intubation: case report and review of the literature. Eur J Pediatr. 165(9):660-1, 2006
                                            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. 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
                                            12. 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
                                            13. Gustafson LM et al: Spiral CT versus MRI in neonatal airway evaluation. Int J Pediatr Otorhinolaryngol. 52(2):197-201, 2000
                                            14. Beghetti M et al: Double aortic arch. J Pediatr. 133(6):799, 1998
                                            15. Donnelly LF et al: The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol. 168(1):59-62, 1997
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Double aortic arch (DAA)
                                                    • Definitions

                                                      • Congenital aortic arch anomaly related to persistence of both left & right 4th aortic arches

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Fluoroscopic Findings

                                                          • CT Findings

                                                            • MR Findings

                                                              • Ultrasonographic 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. Trobo Marina D et al: Neonatal magnetic resonance imaging in double aortic arch diagnosed prenatally by ultrasound. J Obstet Gynaecol. 36(4):526-8, 2016
                                                                                      2. Gould SW et al: Useful signs for the assessment of vascular rings on cross-sectional imaging. Pediatr Radiol. 45(13):2004-16, 2015
                                                                                      3. Iwaki R et al: Follow-up of persistent tracheal stenosis after surgery for a double aortic arch. World J Pediatr Congenit Heart Surg. 6(3):458-61, 2015
                                                                                      4. Trobo D et al: Prenatal sonographic features of a double aortic arch: literature review and perinatal management. J Ultrasound Med. 34(11):1921-7, 2015
                                                                                      5. Kir M et al: Vascular rings: presentation, imaging strategies, treatment, and outcome. Pediatr Cardiol. 33(4):607-17, 2012
                                                                                      6. Lee EY et al: MDCT evaluation of the prevalence of tracheomalacia in children with mediastinal aortic vascular anomalies. J Thorac Imaging. 23(4):258-65, 2008
                                                                                      7. Alsenaidi K et al: Management and outcomes of double aortic arch in 81 patients. Pediatrics. 118(5):e1336-41, 2006
                                                                                      8. Malik TH et al: The role of magnetic resonance imaging in the assessment of suspected extrinsic tracheobronchial compression due to vascular anomalies. Arch Dis Child. 91(1):52-5, 2006
                                                                                      9. van Woerkum F et al: Aortoesophageal fistula due to double aortic arch and prolonged nasogastric intubation: case report and review of the literature. Eur J Pediatr. 165(9):660-1, 2006
                                                                                      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. 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
                                                                                      12. 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
                                                                                      13. Gustafson LM et al: Spiral CT versus MRI in neonatal airway evaluation. Int J Pediatr Otorhinolaryngol. 52(2):197-201, 2000
                                                                                      14. Beghetti M et al: Double aortic arch. J Pediatr. 133(6):799, 1998
                                                                                      15. Donnelly LF et al: The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol. 168(1):59-62, 1997