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Acute Aortic Syndrome
Santiago Martínez-Jiménez, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Acute aortic syndrome (AAS)
          • Definitions

            • AAS: Aortic dissection, incomplete dissection, penetrating aortic ulcer, intramural hematoma
              • Aortic dissection (AD) or class 1 dissection
                • Separation of aortic media by intimomedial flap that separates false channel from aortic lumen
                • Entrance and reentrance tears (occasionally several communications)
              • Intramural hematoma (IMH) or class 2 dissection
                • Aortic media hemorrhage from spontaneous vasa vasorum rhexis (occasionally trauma)
                • Absent (or very small) entrance tear and reentrance tear
              • Penetrating aortic ulcer (PAU) or class 4 dissection
                • Ulcerated atherosclerotic lesion that penetrates internal elastic lamina
                • Associated with variable amounts of intramural hemorrhage
              • Incomplete aortic dissection (ID) or class 3 dissection
                • a.k.a. subtle or discrete aortic dissection, ~ 7% of all dissections
                • Entrance tear (intimal laceration) with no flap or intramural hematoma
                • If only contained by adventitia (contained aortic rupture), appears as subadventitial hematoma
                • Better outcome compared to classic dissection (lower operative mortality, shorter hospital stay, less frequent transfusions)
            • Rhexis:Rupture of vasa vasorum leading to IMH

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Echocardiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Chiu KW et al: Acute aortic syndrome: CT findings. Clin Radiol. 68(7):741-8, 2013
                                  2. Akin I et al: Penetrating aortic ulcer, intramural hematoma, acute aortic syndrome: when to do what. J Cardiovasc Surg (Torino). 53(1 Suppl 1):83-90, 2012
                                  3. Harris KM et al: Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation. 126(11 Suppl 1):S91-6, 2012
                                  4. Wu MT et al: Intramural blood pools accompanying aortic intramural hematoma: CT appearance and natural course. Radiology. 258(3):705-13, 2011
                                  5. Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010
                                  6. Attia R et al: In patients with acute aortic intramural haematoma is open surgical repair superior to conservative management? Interact Cardiovasc Thorac Surg. 9(5):868-71, 2009
                                  7. Ramanath VS et al: Acute aortic syndromes and thoracic aortic aneurysm. Mayo Clin Proc. 84(5):465-81, 2009
                                  8. Lohan DG et al: MR imaging of the thoracic aorta. Magn Reson Imaging Clin N Am. 16(2):213-34, viii, 2008
                                  9. Smith AD et al: CT imaging for acute aortic syndrome. Cleve Clin J Med. 75(1):7-9, 12, 15-7 passim, 2008
                                  10. Chirillo F et al: Clinical and anatomical characteristics of subtle-discrete dissection of the ascending aorta. Am J Cardiol. 100(8):1314-9, 2007
                                  11. François CJ et al: MRI of the thoracic aorta. Cardiol Clin. 25(1):171-84, vii, 2007
                                  12. Flachskampf FA: Assessment of aortic dissection and hematoma. Semin Cardiothorac Vasc Anesth. 10(1):83-8, 2006
                                  13. Evangelista A et al: Acute intramural hematoma of the aorta: a mystery in evolution. Circulation. 111(8):1063-70, 2005
                                  14. Hartnell GG et al: Aortic fenestration: a why, when, and how-to guide. Radiographics. 25(1):175-89, 2005
                                  15. Ko SF et al: Effects of heart rate on motion artifacts of the aorta on non-ECG-assisted 0.5-sec thoracic MDCT. AJR Am J Roentgenol. 184(4):1225-30, 2005
                                  16. Tsai TT et al: Acute aortic syndromes. Circulation. 112(24):3802-13, 2005
                                  17. Macura KJ et al: Pathogenesis in acute aortic syndromes: aortic dissection, intramural hematoma, and penetrating atherosclerotic aortic ulcer. AJR Am J Roentgenol. 181(2):309-16, 2003
                                  18. Song JK et al: Outcomes of medically treated patients with aortic intramural hematoma. Am J Med. 113(3):181-7, 2002
                                  19. Erbel R et al: Diagnosis and management of aortic dissection. Eur Heart J. 22(18):1642-81, 2001
                                  20. Sawhney NS et al: Aortic intramural hematoma: an increasingly recognized and potentially fatal entity. Chest. 120(4):1340-6, 2001
                                  21. Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000
                                  22. Hayashi H et al: Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics. 20(4):995-1005, 2000
                                  23. Maraj R et al: Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol. 86(6):664-8, 2000
                                  24. Pepi M et al: Rapid diagnosis and management of thoracic aortic dissection and intramural haematoma: a prospective study of advantages of multiplane vs. biplane transoesophageal echocardiography. Eur J Echocardiogr. 1(1):72-9, 2000
                                  25. Svensson LG et al: Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation. 99(10):1331-6, 1999
                                  26. Arita T et al: Abdominal aortic aneurysm: rupture associated with the high-attenuating crescent sign. Radiology. 204(3):765-8, 1997
                                  27. Murray JG et al: Intramural hematoma of the thoracic aorta: MR image findings and their prognostic implications. Radiology. 204(2):349-55, 1997
                                  28. Williams DM et al: The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise. Radiology. 203(1):37-44, 1997
                                  29. Slonim SM et al: True lumen obliteration in complicated aortic dissection: endovascular treatment. Radiology. 201(1):161-6, 1996
                                  30. Burns MA et al: Motion artifact simulating aortic dissection on CT. AJR Am J Roentgenol. 157(3):465-7, 1991
                                  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

                                            • Acute aortic syndrome (AAS)
                                          • Definitions

                                            • AAS: Aortic dissection, incomplete dissection, penetrating aortic ulcer, intramural hematoma
                                              • Aortic dissection (AD) or class 1 dissection
                                                • Separation of aortic media by intimomedial flap that separates false channel from aortic lumen
                                                • Entrance and reentrance tears (occasionally several communications)
                                              • Intramural hematoma (IMH) or class 2 dissection
                                                • Aortic media hemorrhage from spontaneous vasa vasorum rhexis (occasionally trauma)
                                                • Absent (or very small) entrance tear and reentrance tear
                                              • Penetrating aortic ulcer (PAU) or class 4 dissection
                                                • Ulcerated atherosclerotic lesion that penetrates internal elastic lamina
                                                • Associated with variable amounts of intramural hemorrhage
                                              • Incomplete aortic dissection (ID) or class 3 dissection
                                                • a.k.a. subtle or discrete aortic dissection, ~ 7% of all dissections
                                                • Entrance tear (intimal laceration) with no flap or intramural hematoma
                                                • If only contained by adventitia (contained aortic rupture), appears as subadventitial hematoma
                                                • Better outcome compared to classic dissection (lower operative mortality, shorter hospital stay, less frequent transfusions)
                                            • Rhexis:Rupture of vasa vasorum leading to IMH

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • CT Findings

                                                • MR Findings

                                                  • Echocardiographic Findings

                                                    • Imaging Recommendations

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            CLINICAL ISSUES

                                                            • Presentation

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Chiu KW et al: Acute aortic syndrome: CT findings. Clin Radiol. 68(7):741-8, 2013
                                                                  2. Akin I et al: Penetrating aortic ulcer, intramural hematoma, acute aortic syndrome: when to do what. J Cardiovasc Surg (Torino). 53(1 Suppl 1):83-90, 2012
                                                                  3. Harris KM et al: Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation. 126(11 Suppl 1):S91-6, 2012
                                                                  4. Wu MT et al: Intramural blood pools accompanying aortic intramural hematoma: CT appearance and natural course. Radiology. 258(3):705-13, 2011
                                                                  5. Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010
                                                                  6. Attia R et al: In patients with acute aortic intramural haematoma is open surgical repair superior to conservative management? Interact Cardiovasc Thorac Surg. 9(5):868-71, 2009
                                                                  7. Ramanath VS et al: Acute aortic syndromes and thoracic aortic aneurysm. Mayo Clin Proc. 84(5):465-81, 2009
                                                                  8. Lohan DG et al: MR imaging of the thoracic aorta. Magn Reson Imaging Clin N Am. 16(2):213-34, viii, 2008
                                                                  9. Smith AD et al: CT imaging for acute aortic syndrome. Cleve Clin J Med. 75(1):7-9, 12, 15-7 passim, 2008
                                                                  10. Chirillo F et al: Clinical and anatomical characteristics of subtle-discrete dissection of the ascending aorta. Am J Cardiol. 100(8):1314-9, 2007
                                                                  11. François CJ et al: MRI of the thoracic aorta. Cardiol Clin. 25(1):171-84, vii, 2007
                                                                  12. Flachskampf FA: Assessment of aortic dissection and hematoma. Semin Cardiothorac Vasc Anesth. 10(1):83-8, 2006
                                                                  13. Evangelista A et al: Acute intramural hematoma of the aorta: a mystery in evolution. Circulation. 111(8):1063-70, 2005
                                                                  14. Hartnell GG et al: Aortic fenestration: a why, when, and how-to guide. Radiographics. 25(1):175-89, 2005
                                                                  15. Ko SF et al: Effects of heart rate on motion artifacts of the aorta on non-ECG-assisted 0.5-sec thoracic MDCT. AJR Am J Roentgenol. 184(4):1225-30, 2005
                                                                  16. Tsai TT et al: Acute aortic syndromes. Circulation. 112(24):3802-13, 2005
                                                                  17. Macura KJ et al: Pathogenesis in acute aortic syndromes: aortic dissection, intramural hematoma, and penetrating atherosclerotic aortic ulcer. AJR Am J Roentgenol. 181(2):309-16, 2003
                                                                  18. Song JK et al: Outcomes of medically treated patients with aortic intramural hematoma. Am J Med. 113(3):181-7, 2002
                                                                  19. Erbel R et al: Diagnosis and management of aortic dissection. Eur Heart J. 22(18):1642-81, 2001
                                                                  20. Sawhney NS et al: Aortic intramural hematoma: an increasingly recognized and potentially fatal entity. Chest. 120(4):1340-6, 2001
                                                                  21. Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000
                                                                  22. Hayashi H et al: Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics. 20(4):995-1005, 2000
                                                                  23. Maraj R et al: Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol. 86(6):664-8, 2000
                                                                  24. Pepi M et al: Rapid diagnosis and management of thoracic aortic dissection and intramural haematoma: a prospective study of advantages of multiplane vs. biplane transoesophageal echocardiography. Eur J Echocardiogr. 1(1):72-9, 2000
                                                                  25. Svensson LG et al: Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation. 99(10):1331-6, 1999
                                                                  26. Arita T et al: Abdominal aortic aneurysm: rupture associated with the high-attenuating crescent sign. Radiology. 204(3):765-8, 1997
                                                                  27. Murray JG et al: Intramural hematoma of the thoracic aorta: MR image findings and their prognostic implications. Radiology. 204(2):349-55, 1997
                                                                  28. Williams DM et al: The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise. Radiology. 203(1):37-44, 1997
                                                                  29. Slonim SM et al: True lumen obliteration in complicated aortic dissection: endovascular treatment. Radiology. 201(1):161-6, 1996
                                                                  30. Burns MA et al: Motion artifact simulating aortic dissection on CT. AJR Am J Roentgenol. 157(3):465-7, 1991