Overview of acute aortic syndromes
- Warren J Manning, MD
Warren J Manning, MD
- Section Editor — Noninvasive Cardiac Imaging and Stress Testing
- Professor of Medicine and Radiology
- Harvard Medical School
- James H Black, III, MD
James H Black, III, MD
- Associate Professor of Surgery
- Johns Hopkins University
- Section Editors
- Gabriel S Aldea, MD
Gabriel S Aldea, MD
- Section Editor — Cardiac Surgery
- Professor of Surgery
- University of Washington
- Edward Verrier, MD
Edward Verrier, MD
- Section Editor — Cardiac Surgery
- Professor of Surgery
- University of Washington School of Medicine
Acute aortic syndromes include a spectrum of life-threatening aortic conditions. By convention, acute disease is distinguished from chronic disease at an arbitrary time point of two weeks from initial clinical presentation (<24 hours hyperacute, 1 to 14 days acute, 14 to 90 days subacute, and >90 days chronic) and is typically manifest with symptoms. Acute aortic dissection is the most familiar and is defined by a separation of the layers of the aortic wall by an inciting intimal injury. Intimal tear without hematoma, penetrating aortic ulcer, aortic intramural hematoma, and periaortic hematoma are variants of the classically described aortic dissection (figure 1) [1-3]. The changes associated with these are more localized, though they are similar to those of aortic dissection, with penetration and weakening of the aortic wall that predisposes to aortic rupture, which may manifest as a contained periaortic hematoma or free rupture into the chest or abdomen . Each of these conditions can affect the thoracic aorta, abdominal aorta, and, in some cases, both.
An overview of the clinical features, classification, diagnosis, and approach to treatment of acute aortic syndromes including acute aortic dissection, which is also discussed in detail elsewhere, aortic intramural hematoma, and penetrating aortic ulcer are reviewed here. (See "Clinical features and diagnosis of acute aortic dissection" and "Management of acute aortic dissection".)
DEFINITION AND PATHOPHYSIOLOGY
Acute aortic dissection — Acute aortic dissection is defined as a separation of the layers of the aortic wall due to an intimal tear (figure 1). For spontaneous dissection, it is uncertain whether the initiating event is a primary rupture of the intima with secondary dissection of the media or primary hemorrhage within the media and subsequent rupture of the overlying intima . The initial intimal tear can occur in the ascending aorta or descending aorta and occasionally can originate in the abdominal aorta. Blood at high pressure passes through the tear and separates the intima from the media and/or adventitia, creating a true lumen and a false lumen. The dissection can propagate proximally or distally from the initial tear to involve the aortic valve, coronary arteries, or branches of the thoracic or abdominal aorta . Such propagation is responsible for many of the associated clinical features of aortic dissection (acute chest or back pain, neurologic symptoms). A higher mean pressure in the false lumen can cause dynamic or static compression and occlusion of the true lumen with malperfusion of the branches of the aorta, resulting in end-organ ischemia (coronary, cerebral, spinal, extremity, visceral) . Aortic regurgitation, coronary ischemia, and cardiac tamponade can occur if the dissection progresses proximally to involve the aortic valve or the pericardial sac. In addition, multiple communications may form between the true lumen and the false lumen. (See "Clinical features and diagnosis of acute aortic dissection", section on 'Clinical features'.)
Abdominal aortic dissection can occur as an extension to a thoracic aortic dissection with the intimal flap located in the proximal or descending thoracic aorta, or it can occur in isolation [7-13]. Isolated abdominal aortic dissection is reported occasionally and can be due to spontaneous, iatrogenic, or traumatic mechanisms . The infrarenal abdominal aorta is more commonly involved than the suprarenal aorta. In one review of 52 reported cases, the entry site for spontaneous isolated abdominal aortic dissections (SIAADs) most commonly occurred between the renal arteries and inferior mesenteric artery . A concomitant abdominal aortic aneurysm was identified in 40 percent of patients and indicated the need for repair. (See "Management of asymptomatic abdominal aortic aneurysm".)
Intimal tear without hematoma — Intimal tear without hematoma (figure 1) is an uncommon variant of aortic dissection that is characterized by a stellate or linear intimal tear associated with exposure of the underlying aortic media or adventitia. There is no separation of the medial layers or progression .
- Nienaber CA, Fattori R, Mehta RH, et al. Gender-related differences in acute aortic dissection. Circulation 2004; 109:3014.
- Tsai TT, Nienaber CA, Eagle KA. Acute aortic syndromes. Circulation 2005; 112:3802.
- Svensson LG, Labib SB, Eisenhauer AC, Butterly JR. Intimal tear without hematoma: an important variant of aortic dissection that can elude current imaging techniques. Circulation 1999; 99:1331.
- Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol 1984; 53:849.
- Nienaber CA, Eagle KA. Aortic dissection: new frontiers in diagnosis and management: Part I: from etiology to diagnostic strategies. Circulation 2003; 108:628.
- Williams DM, Lee DY, Hamilton BH, et al. The dissected aorta: part III. Anatomy and radiologic diagnosis of branch-vessel compromise. Radiology 1997; 203:37.
- Ivkosić A, Budincević H, Krstonijević Z, et al. Spontaneous isolated dissection of the abdominal aorta. Coll Antropol 2013; 37:1361.
- Tang EL, Chong CS, Narayanan S. Isolated abdominal aortic dissection. BMJ Case Rep 2014; 2014.
- Mózes G, Gloviczki P, Park WM, et al. Spontaneous dissection of the infrarenal abdominal aorta. Semin Vasc Surg 2002; 15:128.
- Farber A, Lauterbach SR, Wagner WH, et al. Spontaneous infrarenal abdominal aortic dissection presenting as claudication: case report and review of the literature. Ann Vasc Surg 2004; 18:4.
- Wall ML, Newman JE, Slaney PL, et al. Isolated dissecting aneurysms of the abdominal aorta and the superior mesenteric artery. A case report and literature review. Ann Vasc Surg 2014; 28:1937.e5.
- Naganuma M, Matsui H, Fushimi K, Yasunaga H. Clinical features of isolated dissections of abdominal aortic branches. Heart Vessels 2016; 31:1006.
- Jawadi N, Bisdas T, Torsello G, et al. Endovascular treatment of isolated abdominal aortic dissections: long-term results. J Endovasc Ther 2014; 21:324.
- Iwasaki H, Shibuya T, Shintani T, et al. Abdominal aortic grafting for spontaneous infrarenal abdominal aortic dissection. Ann Vasc Surg 2010; 24:255.e13.
- Farber A, Wagner WH, Cossman DV, et al. Isolated dissection of the abdominal aorta: clinical presentation and therapeutic options. J Vasc Surg 2002; 36:205.
- Vilacosta I, San Román JA, Ferreirós J, et al. Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. Am Heart J 1997; 134:495.
- Maraj R, Rerkpattanapipat P, Jacobs LE, et al. Meta-analysis of 143 reported cases of aortic intramural hematoma. Am J Cardiol 2000; 86:664.
- Hayashi H, Matsuoka Y, Sakamoto I, et al. Penetrating atherosclerotic ulcer of the aorta: imaging features and disease concept. Radiographics 2000; 20:995.
- Stanson AW, Kazmier FJ, Hollier LH, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations. Ann Vasc Surg 1986; 1:15.
- Shennan T. Dissecting aneurysms. Medical Research Council. Special Report. No. 193. 1934.
- Ganaha F, Miller DC, Sugimoto K, et al. Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. Circulation 2002; 106:342.
- HIRST AE Jr, BARBOUR BH. Dissecting aneurysm with hemopericardium; report of a case with healing. N Engl J Med 1958; 258:116.
- Mohr-Kahaly S, Erbel R, Kearney P, et al. Aortic intramural hemorrhage visualized by transesophageal echocardiography: findings and prognostic implications. J Am Coll Cardiol 1994; 23:658.
- Nienaber CA, von Kodolitsch Y, Petersen B, et al. Intramural hemorrhage of the thoracic aorta. Diagnostic and therapeutic implications. Circulation 1995; 92:1465.
- Song JK, Kim HS, Kang DH, et al. Different clinical features of aortic intramural hematoma versus dissection involving the ascending aorta. J Am Coll Cardiol 2001; 37:1604.
- Goldberg JB, Kim JB, Sundt TM. Current understandings and approach to the management of aortic intramural hematomas. Semin Thorac Cardiovasc Surg 2014; 26:123.
- GORE I. Pathogenesis of dissecting aneurysm of the aorta. AMA Arch Pathol 1952; 53:142.
- Uchida K, Imoto K, Karube N, et al. Intramural haematoma should be referred to as thrombosed-type aortic dissection. Eur J Cardiothorac Surg 2013; 44:366.
- Park KH, Lim C, Choi JH, et al. Prevalence of aortic intimal defect in surgically treated acute type A intramural hematoma. Ann Thorac Surg 2008; 86:1494.
- Kitai T, Kaji S, Yamamuro A, et al. Detection of intimal defect by 64-row multidetector computed tomography in patients with acute aortic intramural hematoma. Circulation 2011; 124:S174.
- Uchida K, Imoto K, Takahashi M, et al. Pathologic characteristics and surgical indications of superacute type A intramural hematoma. Ann Thorac Surg 2005; 79:1518.
- Harris KM, Braverman AC, Eagle KA, et al. Acute aortic intramural hematoma: an analysis from the International Registry of Acute Aortic Dissection. Circulation 2012; 126:S91.
- Estrera A, Miller C 3rd, Lee TY, et al. Acute type A intramural hematoma: analysis of current management strategy. Circulation 2009; 120:S287.
- Tittle SL, Lynch RJ, Cole PE, et al. Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta. J Thorac Cardiovasc Surg 2002; 123:1051.
- Evangelista A, Mukherjee D, Mehta RH, et al. Acute intramural hematoma of the aorta: a mystery in evolution. Circulation 2005; 111:1063.
- Mukherjee D, Evangelista A, Nienaber CA, et al. Implications of periaortic hematoma in patients with acute aortic dissection (from the International Registry of Acute Aortic Dissection). Am J Cardiol 2005; 96:1734.
- DEBAKEY ME, HENLY WS, COOLEY DA, et al. SURGICAL MANAGEMENT OF DISSECTING ANEURYSMS OF THE AORTA. J Thorac Cardiovasc Surg 1965; 49:130.
- Daily PO, Trueblood HW, Stinson EB, et al. Management of acute aortic dissections. Ann Thorac Surg 1970; 10:237.
- Pape LA, Awais M, Woznicki EM, et al. Presentation, Diagnosis, and Outcomes of Acute Aortic Dissection: 17-Year Trends From the International Registry of Acute Aortic Dissection. J Am Coll Cardiol 2015; 66:350.
- Khoynezhad A, Walot I, Kruse MJ, et al. Distribution of intimomedial tears in patients with type B aortic dissection. J Vasc Surg 2010; 52:562.
- Song JK, Kim HS, Song JM, et al. Outcomes of medically treated patients with aortic intramural hematoma. Am J Med 2002; 113:181.
- Nienaber CA, Eagle KA. Aortic dissection: new frontiers in diagnosis and management: Part II: therapeutic management and follow-up. Circulation 2003; 108:772.
- Moizumi Y, Komatsu T, Motoyoshi N, Tabayashi K. Clinical features and long-term outcome of type A and type B intramural hematoma of the aorta. J Thorac Cardiovasc Surg 2004; 127:421.
- Eggebrecht H, Plicht B, Kahlert P, Erbel R. Intramural hematoma and penetrating ulcers: indications to endovascular treatment. Eur J Vasc Endovasc Surg 2009; 38:659.
- Coady MA, Rizzo JA, Hammond GL, et al. Penetrating ulcer of the thoracic aorta: what is it? How do we recognize it? How do we manage it? J Vasc Surg 1998; 27:1006.
- Cho KR, Stanson AW, Potter DD, et al. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch. J Thorac Cardiovasc Surg 2004; 127:1393.
- Nathan DP, Boonn W, Lai E, et al. Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease. J Vasc Surg 2012; 55:10.
- LeMaire SA, Russell L. Epidemiology of thoracic aortic dissection. Nat Rev Cardiol 2011; 8:103.
- Bickerstaff LK, Pairolero PC, Hollier LH, et al. Thoracic aortic aneurysms: a population-based study. Surgery 1982; 92:1103.
- Mészáros I, Mórocz J, Szlávi J, et al. Epidemiology and clinicopathology of aortic dissection. Chest 2000; 117:1271.
- Clouse WD, Hallett JW Jr, Schaff HV, et al. Acute aortic dissection: population-based incidence compared with degenerative aortic aneurysm rupture. Mayo Clin Proc 2004; 79:176.
- Hagan PG, Nienaber CA, Isselbacher EM, et al. The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. JAMA 2000; 283:897.
- Spittell PC, Spittell JA Jr, Joyce JW, et al. Clinical features and differential diagnosis of aortic dissection: experience with 236 cases (1980 through 1990). Mayo Clin Proc 1993; 68:642.
- Januzzi JL, Isselbacher EM, Fattori R, et al. Characterizing the young patient with aortic dissection: results from the International Registry of Aortic Dissection (IRAD). J Am Coll Cardiol 2004; 43:665.
- Evangelista A, Eagle KA. Is the optimal management of acute type a aortic intramural hematoma evolving? Circulation 2009; 120:2029.
- Pelzel JM, Braverman AC, Hirsch AT, Harris KM. International heterogeneity in diagnostic frequency and clinical outcomes of ascending aortic intramural hematoma. J Am Soc Echocardiogr 2007; 20:1260.
- Kitai T, Kaji S, Yamamuro A, et al. Clinical outcomes of medical therapy and timely operation in initially diagnosed type a aortic intramural hematoma: a 20-year experience. Circulation 2009; 120:S292.
- Farooq MM, Kling K, Yamini D, et al. Penetrating ulceration of the infrarenal aorta: case reports of an embolic and an asymptomatic lesion. Ann Vasc Surg 2001; 15:255.
- Ryan A, McCook B, Sholosh B, et al. Acute intramural hematoma of the aorta as a cause of positive FDG PET/CT. Clin Nucl Med 2007; 32:729.
- Alexanderson-Rosas E, Martinez-Aguilar MM, Jimenez-Santos M, et al. Incidental aortic dissection diagnostic: Importance of hybrid imaging with PET/CT. J Nucl Cardiol 2015; 22:849.
- Etz CD, Kari FA, Mueller CS, et al. The collateral network concept: a reassessment of the anatomy of spinal cord perfusion. J Thorac Cardiovasc Surg 2011; 141:1020.
- von Kodolitsch Y, Csösz SK, Koschyk DH, et al. Intramural hematoma of the aorta: predictors of progression to dissection and rupture. Circulation 2003; 107:1158.
- Kang DH, Song JK, Song MG, et al. Clinical and echocardiographic outcomes of aortic intramural hemorrhage compared with acute aortic dissection. Am J Cardiol 1998; 81:202.
- Suzuki T, Distante A, Zizza A, et al. Diagnosis of acute aortic dissection by D-dimer: the International Registry of Acute Aortic Dissection Substudy on Biomarkers (IRAD-Bio) experience. Circulation 2009; 119:2702.
- von Kodolitsch Y, Nienaber CA, Dieckmann C, et al. Chest radiography for the diagnosis of acute aortic syndrome. Am J Med 2004; 116:73.
- Keren A, Kim CB, Hu BS, et al. Accuracy of biplane and multiplane transesophageal echocardiography in diagnosis of typical acute aortic dissection and intramural hematoma. J Am Coll Cardiol 1996; 28:627.
- Nienaber CA. The role of imaging in acute aortic syndromes. Eur Heart J Cardiovasc Imaging 2013; 14:15.
- Erbel R, Alfonso F, Boileau C, et al. Diagnosis and management of aortic dissection. Eur Heart J 2001; 22:1642.
- Chae MK, Kim EK, Jung KY, et al. Triple rule-out computed tomography for risk stratification of patients with acute chest pain. J Cardiovasc Comput Tomogr 2016; 10:291.
- Hiratzka LF, Bakris GL, Beckman JA, 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: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation 2010; 121:e266.
- Steuer J, Björck M, Mayer D, et al. Distinction between acute and chronic type B aortic dissection: is there a sub-acute phase? Eur J Vasc Endovasc Surg 2013; 45:627.
- Chan KK, Lai P, Wright JM. First-line beta-blockers versus other antihypertensive medications for chronic type B aortic dissection. Cochrane Database Syst Rev 2014; :CD010426.
- Trimarchi S, Eagle KA, Nienaber CA, et al. Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD). Circulation 2010; 122:1283.
- Januzzi JL, Movsowitz HD, Choi J, et al. Significance of recurrent pain in acute type B aortic dissection. Am J Cardiol 2001; 87:930.
- Di Eusanio M, Trimarchi S, Patel HJ, et al. Clinical presentation, management, and short-term outcome of patients with type A acute dissection complicated by mesenteric malperfusion: observations from the International Registry of Acute Aortic Dissection. J Thorac Cardiovasc Surg 2013; 145:385.
- Pacini D, Leone A, Belotti LM, et al. Acute type A aortic dissection: significance of multiorgan malperfusion. Eur J Cardiothorac Surg 2013; 43:820.
- Geirsson A, Szeto WY, Pochettino A, et al. Significance of malperfusion syndromes prior to contemporary surgical repair for acute type A dissection: outcomes and need for additional revascularizations. Eur J Cardiothorac Surg 2007; 32:255.
- Kaji S, Nishigami K, Akasaka T, et al. Prediction of progression or regression of type A aortic intramural hematoma by computed tomography. Circulation 1999; 100:II281.
- Sawhney NS, DeMaria AN, Blanchard DG. Aortic intramural hematoma: an increasingly recognized and potentially fatal entity. Chest 2001; 120:1340.
- Isselbacher EM. Intramural hematoma of the aorta: should we let down our guard? Am J Med 2002; 113:244.
- Song JK, Yim JH, Ahn JM, et al. Outcomes of patients with acute type a aortic intramural hematoma. Circulation 2009; 120:2046.
- Ho HH, Cheung CW, Jim MH, et al. Type A aortic intramural hematoma: clinical features and outcomes in Chinese patients. Clin Cardiol 2011; 34:E1.
- Kaji S, Akasaka T, Katayama M, et al. Long-term prognosis of patients with type B aortic intramural hematoma. Circulation 2003; 108 Suppl 1:II307.
- Song JK, Kang DH, Lim TH, et al. Different remodeling of descending thoracic aorta after acute event in aortic intramural hemorrhage versus aortic dissection. Am J Cardiol 1999; 83:937.
- Sueyoshi E, Imada T, Sakamoto I, et al. Analysis of predictive factors for progression of type B aortic intramural hematoma with computed tomography. J Vasc Surg 2002; 35:1179.
- Sueyoshi E, Sakamoto I, Fukuda M, et al. Long-term outcome of type B aortic intramural hematoma: comparison with classic aortic dissection treated by the same therapeutic strategy. Ann Thorac Surg 2004; 78:2112.
- Bosma MS, Quint LE, Williams DM, et al. Ulcerlike projections developing in noncommunicating aortic dissections: CT findings and natural history. AJR Am J Roentgenol 2009; 193:895.
- Demers P, Miller DC, Mitchell RS, et al. Stent-graft repair of penetrating atherosclerotic ulcers in the descending thoracic aorta: mid-term results. Ann Thorac Surg 2004; 77:81.
- DEFINITION AND PATHOPHYSIOLOGY
- Acute aortic dissection
- - Intimal tear without hematoma
- - Iatrogenic or traumatic dissection
- Penetrating aortic ulcer
- Aortic intramural hematoma
- Periaortic hematoma
- EPIDEMIOLOGY AND RISK FACTORS
- CLINICAL FEATURES
- Differential diagnosis
- Treatment overview
- Acute medical management
- - Anti-impulse therapy
- - Pain control
- Progression and subsequent treatment
- - Ascending aorta (type A)
- - Descending aorta (type B)
- Open surgical and endovascular techniques
- SUMMARY AND RECOMMENDATIONS