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Acute Aortic Syndromes
Santiago Martínez-Jiménez, MD; Julia Alegría, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Acute aortic syndrome (AAS): Aortic dissection (AD), incomplete dissection (ID), penetrating aortic ulcer (PAU), intramural hematoma (IMH)
              • AD or class 1 dissection
                • Aortic media separated by intimomedial flap between false and true aortic lumina
                • Entrance and re-entrance tears (occasionally several communications)
              • IMH or class 2 dissection
                • Aortic media hemorrhage from spontaneous vasa vasorum rhexis (occasionally trauma)
                • Absent (or very small) entrance and re-entrance tears
              • ID or class 3 dissection
                • Also known as subtle or discrete AD, ~ 7% of all dissections
                • Entrance tear (intimal laceration) with no flap or IMH
                • 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)
              • PAU or class 4 dissection
                • Ulcerated atherosclerotic lesion that penetrates internal elastic lamina
                • Associated with variable amounts of intramural hemorrhage
            • Rhexis: Rupture of vasa vasorum leading to IMH
            • Focal aortic projection (FAP): Intrinsic contrast collection within intramural or subadventitial hematoma
              • Intramural blood pool (IBP) (branch pseudoaneurysm); benign, can be observed
              • PAU
              • Ulcer-like projection (ULP); treated as PAU

          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

                                  • Essential Imaging Report Content

                                    Selected References

                                    1. Bossone E et al: Imaging and biomarkers in acute aortic syndromes: diagnostic and prognostic implications. Curr Probl Cardiol. 46(3):100654, 2021
                                    2. Carroll BJ et al: Imaging for acute aortic syndromes. Heart. 106(3):182-9, 2019
                                    3. Sailer AM et al: Computed tomography imaging features in acute uncomplicated stanford type-B aortic dissection predict late adverse events. Circ Cardiovasc Imaging. 10(4), 2017
                                    4. Gutschow SE et al: Emerging concepts in intramural hematoma imaging. Radiographics. 36(3):660-74, 2016
                                    5. Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 121(13):e266-369, 2010. Erratum in: Circulation. 122(4):e410, 2010
                                    6. Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010
                                    7. 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
                                    8. Ramanath VS et al: Acute aortic syndromes and thoracic aortic aneurysm. Mayo Clin Proc. 84(5):465-81, 2009
                                    9. Lohan DG et al: MR imaging of the thoracic aorta. Magn Reson Imaging Clin N Am. 16(2):213-34, viii, 2008
                                    10. Smith AD et al: CT imaging for acute aortic syndrome. Cleve Clin J Med. 75(1):7-9, 12, 15-7 passim, 2008
                                    11. Chirillo F et al: Clinical and anatomical characteristics of subtle-discrete dissection of the ascending aorta. Am J Cardiol. 100(8):1314-9, 2007
                                    12. François CJ et al: MRI of the thoracic aorta. Cardiol Clin. 25(1):171-84, vii, 2007
                                    13. Flachskampf FA: Assessment of aortic dissection and hematoma. Semin Cardiothorac Vasc Anesth. 10(1):83-8, 2006
                                    14. Evangelista A et al: Acute intramural hematoma of the aorta: a mystery in evolution. Circulation. 111(8):1063-70, 2005
                                    15. Hartnell GG et al: Aortic fenestration: a why, when, and how-to guide. Radiographics. 25(1):175-89, 2005
                                    16. 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
                                    17. Tsai TT et al: Acute aortic syndromes. Circulation. 112(24):3802-13, 2005
                                    18. 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
                                    19. Song JK et al: Outcomes of medically treated patients with aortic intramural hematoma. Am J Med. 113(3):181-7, 2002
                                    20. Erbel R et al: Diagnosis and management of aortic dissection. Eur Heart J. 22(18):1642-81, 2001
                                    21. Sawhney NS et al: Aortic intramural hematoma: an increasingly recognized and potentially fatal entity. Chest. 120(4):1340-6, 2001
                                    22. Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000
                                    23. Hayashi H et al: Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics. 20(4):995-1005, 2000
                                    24. Maraj R et al: Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol. 86(6):664-8, 2000
                                    25. 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
                                    26. 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
                                    27. Arita T et al: Abdominal aortic aneurysm: rupture associated with the high-attenuating crescent sign. Radiology. 204(3):765-8, 1997
                                    28. Murray JG et al: Intramural hematoma of the thoracic aorta: MR image findings and their prognostic implications. Radiology. 204(2):349-55, 1997
                                    29. Williams DM et al: The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise. Radiology. 203(1):37-44, 1997
                                    30. Slonim SM et al: True lumen obliteration in complicated aortic dissection: endovascular treatment. Radiology. 201(1):161-6, 1996
                                    31. 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

                                            • Definitions

                                              • Acute aortic syndrome (AAS): Aortic dissection (AD), incomplete dissection (ID), penetrating aortic ulcer (PAU), intramural hematoma (IMH)
                                                • AD or class 1 dissection
                                                  • Aortic media separated by intimomedial flap between false and true aortic lumina
                                                  • Entrance and re-entrance tears (occasionally several communications)
                                                • IMH or class 2 dissection
                                                  • Aortic media hemorrhage from spontaneous vasa vasorum rhexis (occasionally trauma)
                                                  • Absent (or very small) entrance and re-entrance tears
                                                • ID or class 3 dissection
                                                  • Also known as subtle or discrete AD, ~ 7% of all dissections
                                                  • Entrance tear (intimal laceration) with no flap or IMH
                                                  • 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)
                                                • PAU or class 4 dissection
                                                  • Ulcerated atherosclerotic lesion that penetrates internal elastic lamina
                                                  • Associated with variable amounts of intramural hemorrhage
                                              • Rhexis: Rupture of vasa vasorum leading to IMH
                                              • Focal aortic projection (FAP): Intrinsic contrast collection within intramural or subadventitial hematoma
                                                • Intramural blood pool (IBP) (branch pseudoaneurysm); benign, can be observed
                                                • PAU
                                                • Ulcer-like projection (ULP); treated as PAU

                                            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

                                                                    • Essential Imaging Report Content

                                                                      Selected References

                                                                      1. Bossone E et al: Imaging and biomarkers in acute aortic syndromes: diagnostic and prognostic implications. Curr Probl Cardiol. 46(3):100654, 2021
                                                                      2. Carroll BJ et al: Imaging for acute aortic syndromes. Heart. 106(3):182-9, 2019
                                                                      3. Sailer AM et al: Computed tomography imaging features in acute uncomplicated stanford type-B aortic dissection predict late adverse events. Circ Cardiovasc Imaging. 10(4), 2017
                                                                      4. Gutschow SE et al: Emerging concepts in intramural hematoma imaging. Radiographics. 36(3):660-74, 2016
                                                                      5. Hiratzka LF et al: 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 121(13):e266-369, 2010. Erratum in: Circulation. 122(4):e410, 2010
                                                                      6. Vilacosta I et al: Acute aortic syndrome: a new look at an old conundrum. Postgrad Med J. 86(1011):52-61, 2010
                                                                      7. 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
                                                                      8. Ramanath VS et al: Acute aortic syndromes and thoracic aortic aneurysm. Mayo Clin Proc. 84(5):465-81, 2009
                                                                      9. Lohan DG et al: MR imaging of the thoracic aorta. Magn Reson Imaging Clin N Am. 16(2):213-34, viii, 2008
                                                                      10. Smith AD et al: CT imaging for acute aortic syndrome. Cleve Clin J Med. 75(1):7-9, 12, 15-7 passim, 2008
                                                                      11. Chirillo F et al: Clinical and anatomical characteristics of subtle-discrete dissection of the ascending aorta. Am J Cardiol. 100(8):1314-9, 2007
                                                                      12. François CJ et al: MRI of the thoracic aorta. Cardiol Clin. 25(1):171-84, vii, 2007
                                                                      13. Flachskampf FA: Assessment of aortic dissection and hematoma. Semin Cardiothorac Vasc Anesth. 10(1):83-8, 2006
                                                                      14. Evangelista A et al: Acute intramural hematoma of the aorta: a mystery in evolution. Circulation. 111(8):1063-70, 2005
                                                                      15. Hartnell GG et al: Aortic fenestration: a why, when, and how-to guide. Radiographics. 25(1):175-89, 2005
                                                                      16. 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
                                                                      17. Tsai TT et al: Acute aortic syndromes. Circulation. 112(24):3802-13, 2005
                                                                      18. 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
                                                                      19. Song JK et al: Outcomes of medically treated patients with aortic intramural hematoma. Am J Med. 113(3):181-7, 2002
                                                                      20. Erbel R et al: Diagnosis and management of aortic dissection. Eur Heart J. 22(18):1642-81, 2001
                                                                      21. Sawhney NS et al: Aortic intramural hematoma: an increasingly recognized and potentially fatal entity. Chest. 120(4):1340-6, 2001
                                                                      22. Batra P et al: Pitfalls in the diagnosis of thoracic aortic dissection at CT angiography. Radiographics. 20(2):309-20, 2000
                                                                      23. Hayashi H et al: Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics. 20(4):995-1005, 2000
                                                                      24. Maraj R et al: Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol. 86(6):664-8, 2000
                                                                      25. 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
                                                                      26. 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
                                                                      27. Arita T et al: Abdominal aortic aneurysm: rupture associated with the high-attenuating crescent sign. Radiology. 204(3):765-8, 1997
                                                                      28. Murray JG et al: Intramural hematoma of the thoracic aorta: MR image findings and their prognostic implications. Radiology. 204(2):349-55, 1997
                                                                      29. Williams DM et al: The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise. Radiology. 203(1):37-44, 1997
                                                                      30. Slonim SM et al: True lumen obliteration in complicated aortic dissection: endovascular treatment. Radiology. 201(1):161-6, 1996
                                                                      31. Burns MA et al: Motion artifact simulating aortic dissection on CT. AJR Am J Roentgenol. 157(3):465-7, 1991