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Interrupted/Hypoplastic Aorta
Harold Goerne, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Interrupted aortic arch (IAA)
              • Hypoplastic aortic arch (HAA)
            • Synonyms

              • Atresia of aortic arch
            • Definitions

              • IAA
                • Complete luminal and anatomical wall discontinuity between ascending aorta and descending aorta
                  • Not true interruption, as there is fibrotic continuity between blind ends
              • HAA
                • Tubular narrowing of aortic arch  without luminal discontinuity

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. LaPar DJ et al: Surgical considerations in interrupted aortic arch. Semin Cardiothorac Vasc Anesth. 22(3):278-84, 2018
                                    2. Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017
                                    3. Goudar SP et al: Echocardiography of coarctation of the aorta, aortic arch hypoplasia, and arch interruption: strategies for evaluation of the aortic arch. Cardiol Young. 26(8):1553-1562, 2016
                                    4. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                    5. Kimura-Hayama ET et al: Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 30(1):79-98, 2010
                                    6. Dillman JR et al: Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 190(6):1467-74, 2008
                                    7. Yang DH et al: Multislice CT angiography of interrupted aortic arch. Pediatr Radiol. 38(1):89-100, 2008
                                    8. Loffredo CA et al: Interrupted aortic arch: an epidemiologic study. Teratology. 61(5):368-75, 2000
                                    9. Kaulitz R et al: Echocardiographic assessment of interrupted aortic arch. Cardiol Young. 9(6):562-71, 1999
                                    10. Reardon MJ et al: Interrupted aortic arch: brief review and summary of an eighteen-year experience. Tex Heart Inst J. 11(3):250-9, 1984
                                    11. CELORIA GC et al: Congenital absence of the aortic arch. Am Heart J. 58:407-13, 1959
                                    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

                                                • Interrupted aortic arch (IAA)
                                                • Hypoplastic aortic arch (HAA)
                                              • Synonyms

                                                • Atresia of aortic arch
                                              • Definitions

                                                • IAA
                                                  • Complete luminal and anatomical wall discontinuity between ascending aorta and descending aorta
                                                    • Not true interruption, as there is fibrotic continuity between blind ends
                                                • HAA
                                                  • Tubular narrowing of aortic arch  without luminal discontinuity

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • MR Findings

                                                    • Ultrasonographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Staging, Grading, & Classification

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. LaPar DJ et al: Surgical considerations in interrupted aortic arch. Semin Cardiothorac Vasc Anesth. 22(3):278-84, 2018
                                                                      2. Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017
                                                                      3. Goudar SP et al: Echocardiography of coarctation of the aorta, aortic arch hypoplasia, and arch interruption: strategies for evaluation of the aortic arch. Cardiol Young. 26(8):1553-1562, 2016
                                                                      4. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                                                      5. Kimura-Hayama ET et al: Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 30(1):79-98, 2010
                                                                      6. Dillman JR et al: Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 190(6):1467-74, 2008
                                                                      7. Yang DH et al: Multislice CT angiography of interrupted aortic arch. Pediatr Radiol. 38(1):89-100, 2008
                                                                      8. Loffredo CA et al: Interrupted aortic arch: an epidemiologic study. Teratology. 61(5):368-75, 2000
                                                                      9. Kaulitz R et al: Echocardiographic assessment of interrupted aortic arch. Cardiol Young. 9(6):562-71, 1999
                                                                      10. Reardon MJ et al: Interrupted aortic arch: brief review and summary of an eighteen-year experience. Tex Heart Inst J. 11(3):250-9, 1984
                                                                      11. CELORIA GC et al: Congenital absence of the aortic arch. Am Heart J. 58:407-13, 1959