[Skip to Content]
[Skip to Content Landing]

Acute Aortic Dissection and Intramural HematomaA Systematic Review

Educational Objective
To review the pathophysiology, presentation, diagnosis and treatment of acute aortic dissection and intramural hematoma.
1 Credit CME
Abstract

Importance  Acute aortic syndrome (AAS), a potentially fatal pathologic process within the aortic wall, should be suspected in patients presenting with severe thoracic pain and hypertension. AAS, including aortic dissection (approximately 90% of cases) and intramural hematoma, may be complicated by poor perfusion, aneurysm, or uncontrollable pain and hypertension. AAS is uncommon (approximately 3.5-6.0 per 100 000 patient-years) but rapid diagnosis is imperative as an emergency surgical procedure is frequently necessary.

Objective  To systematically review the current evidence on diagnosis and treatment of AAS.

Evidence Review  Searches of MEDLINE, EMBASE, and the Cochrane Register of Controlled Trials for articles on diagnosis and treatment of AAS from June 1994 to January 29, 2016, were performed. Only clinical trials and prospective observational studies of 10 or more patients were included. Eighty-two studies (2 randomized clinical trials and 80 observational) describing 57 311 patients were reviewed.

Findings  Chest or back pain was the most commonly reported presenting symptom of AAS (61.6%-84.8%). Patients were typically aged 60 to 70 years, male (50%-81%), and had hypertension (45%-100%). Sensitivities of computerized tomography and magnetic resonance imaging for diagnosis of AAS were 100% and 95% to 100%, respectively. Transesophageal echocardiography was 86% to 100% sensitive, whereas D-dimer was 51.7% to 100% sensitive and 32.8% to 89.2% specific among 6 studies (n = 876). An immediate open surgical procedure is needed for dissection of the ascending aorta, given the high mortality (26%-58%) and proximity to the aortic valve and great vessels (with potential for dissection complications such as tamponade). An RCT comparing endovascular surgical procedure to medical management for uncomplicated AAS in the descending aorta (n = 61) revealed no dissection-related deaths in either group. Endovascular surgical procedure was better than medical treatment (97% vs 43%, P < .001) for the primary end point of “favorable aortic remodeling” (false lumen thrombosis and no aortic dilation or rupture). The remaining evidence on therapies was observational, introducing significant selection bias.

Conclusions and Relevance  Because of the high mortality rate, AAS should be considered and diagnosed promptly in patients presenting with acute chest or back pain and high blood pressure. Computerized tomography, magnetic resonance imaging, and transesophageal echocardiography are reliable tools for diagnosing AAS. Available data suggest that open surgical repair is optimal for treating type A (ascending aorta) AAS, whereas thoracic endovascular aortic repair may be optimal for treating type B (descending aorta) AAS. However, evidence is limited by the paucity of randomized trials.

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ from JAMA Network is your new home for CME and MOC from a source you trust. Earn AMA PRA Category 1 CME Credit™ from relevant articles, audio, and Clinical Challenge image quizzes, explore interactives and videos, and – depending on your specialty or state – have your MOC points automatically transferred to the relevant board. Learn more about CME

Article Information

Corresponding Author: Firas F. Mussa, MD, MS, Division of Vascular Surgery, Department of Surgery, Columbia University Medical Center, 161 Fort Washington Ave, Herbert Irving Pavilion, Fifth Floor, New York, NY 10032 (ffm2110@cumc.columbia.edu).

Author Contributions: Drs Mussa and Horton had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Study concept and design: Mussa, Horton, Moridzadeh, Trimarchi, Eagle.

Acquisition, analysis, or interpretation of data: Horton, Moridzadeh, Nicholson, Trimarchi.

Drafting of the manuscript: Horton, Moridzadeh, Nicholson.

Critical revision of the manuscript for important intellectual content: Mussa, Horton, Moridzadeh, Trimarchi, Eagle.

Statistical analysis: Mussa, Horton.

Obtained funding: Mussa.

Administrative, technical, or material support: Mussa, Nicholson.

Study supervision: Mussa, Trimarchi, Eagle.

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr Trimarchi reports grants and personal fees from Gore and Medtronic. Dr Eagle reports grants from Gore, Medtronic, and Terumo. No other disclosures were reported.

Additional Contributions: We thank Mr G. Craig Wood, MS, for assistance with compilation and organization of the data. We also thank the contributing centers of the International Registry of Aortic Dissection (IRAD) and the coordinating center at the University of Michigan. None of these contributors received compensation.

References
1.
Vilacosta  I, Román  JA.  Acute aortic syndrome.  Heart. 2001;85(4):365-368.PubMedGoogle ScholarCrossref
2.
Clouse  WD, Hallett  JW  Jr, Schaff  HV,  et al.  Acute aortic dissection.  Mayo Clin Proc. 2004;79(2):176-180.PubMedGoogle ScholarCrossref
3.
Howard  DP, Banerjee  A, Fairhead  JF, Perkins  J, Silver  LE, Rothwell  PM; Oxford Vascular Study.  Population-based study of incidence and outcome of acute aortic dissection and premorbid risk factor control.  Circulation. 2013;127(20):2031-2037.PubMedGoogle ScholarCrossref
4.
Olsson  C, Thelin  S, Ståhle  E, Ekbom  A, Granath  F.  Thoracic aortic aneurysm and dissection.  Circulation. 2006;114(24):2611-2618.PubMedGoogle ScholarCrossref
5.
Harris  KM, Braverman  AC, Eagle  KA,  et al Acute aortic intramural hematoma.  Circulation.2012;126(11 suppl 1):S91-S96. PubMedGoogle ScholarCrossref
6.
Ganaha  F, Miller  DC, Sugimoto  K,  et al.  Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer.  Circulation.2002;106(3):342-348. PubMedGoogle ScholarCrossref
7.
Evangelista  A, Mukherjee  D, Mehta  RH,  et al.  Acute intramural hematoma of the aorta.  Circulation. 2005;111(8):1063-1070.PubMedGoogle ScholarCrossref
8.
Nienaber  CA, von Kodolitsch  Y, Petersen  B,  et al.  Intramural hemorrhage of the thoracic aorta.  Circulation. 1995;92(6):1465-1472.PubMedGoogle ScholarCrossref
9.
Tsai  TT, Nienaber  CA, Eagle  KA.  Acute aortic syndromes.  Circulation. 2005;112(24):3802-3813.PubMedGoogle ScholarCrossref
10.
Daily  PO, Trueblood  HW, Stinson  EB, Wuerflein  RD, Shumway  NE.  Management of acute aortic dissections.  Ann Thorac Surg. 1970;10(3):237-247.PubMedGoogle ScholarCrossref
11.
Debakey  ME, Henly  WS, Cooley  DA, Morris  GC  Jr, Crawford  ES, Beall  AC  Jr.  Surgical management of dissecting aneurysms of the aorta.  J Thorac Cardiovasc Surg. 1965;49:130-149.PubMedGoogle Scholar
12.
Liberati  A, Altman  DG, Tetzlaff  J,  et al.  The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions.  BMJ. 2009;339:b2700.PubMedGoogle ScholarCrossref
13.
Higgins  JPGS, ed.  Cochrane Handbook for Systematic Review of Interventions. Chichester, UK: John Wiley and Sons; 2008.
14.
Phillips  B, Ball  C, Sackett  D,  et al.  Oxford Centre for Evidence-based Medicine—levels of evidence. http://www.cebm.net/oxford-centre-evidence-based-medicine-levels-evidence-march-2009/. Accessed July 22, 2016.
15.
Brunkwall  J, Kasprzak  P, Verhoeven  E,  et al.  Endovascular repair of acute uncomplicated aortic type B dissection promotes aortic remodelling [correction appears in Eur J Vasc Endovasc Surg. 2015;50(1):130].  Eur J Vasc Endovasc Surg. 2014;48(3):285-291.PubMedGoogle ScholarCrossref
16.
Nienaber  CA, Kische  S, Rousseau  H,  et al.  Endovascular repair of type B aortic dissection.  Circ Cardiovasc Interv. 2013;6(4):407-416.PubMedGoogle ScholarCrossref
17.
Falconi  M, Oberti  P, Krauss  J,  et al.  Different clinical features of aortic intramural hematoma versus dissection involving the descending thoracic aorta.  Echocardiography. 2005;22(8):629-635.PubMedGoogle ScholarCrossref
18.
Hagan  PG, Nienaber  CA, Isselbacher  EM,  et al.  The International Registry of Acute Aortic Dissection (IRAD).  JAMA. 2000;283(7):897-903.PubMedGoogle ScholarCrossref
19.
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(3):627-636.PubMedGoogle ScholarCrossref
20.
Landenhed  M, Engström  G, Gottsäter  A,  et al.  Risk profiles for aortic dissection and ruptured or surgically treated aneurysms.  J Am Heart Assoc. 2015;4(1):e001513.PubMedGoogle ScholarCrossref
21.
Motoyoshi  N, Moizumi  Y, Komatsu  T, Tabayashi  K.  Intramural hematoma and dissection involving ascending aorta.  Eur J Cardiothorac Surg. 2003;24(2):237-242.PubMedGoogle ScholarCrossref
22.
Tsai  TT, Trimarchi  S, Nienaber  CA.  Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).  Eur J Vasc Endovasc Surg. 2009;37(2):149-159.PubMedGoogle ScholarCrossref
23.
Vagnarelli  F, Corsini  A, Lorenzini  M,  et al.  Acute heart failure in patients with acute aortic syndrome.  Eur J Heart Fail. 2015;17(9):917-924.PubMedGoogle ScholarCrossref
24.
Nienaber  CA, Fattori  R, Mehta  RH,  et al.  Gender-related differences in acute aortic dissection.  Circulation. 2004;109(24):3014-3021.PubMedGoogle ScholarCrossref
25.
Bossone  E, Pyeritz  RE, O’Gara  P,  et al.  Acute aortic dissection in blacks.  Am J Med. 2013;126(10):909-915.PubMedGoogle ScholarCrossref
26.
Dean  JH, Woznicki  EM, O’Gara  P,  et al.  Cocaine-related aortic dissection.  Am J Med. 2014;127(9):878-885.PubMedGoogle ScholarCrossref
27.
Harris  KM, Strauss  CE, Eagle  KA,  et al.  Correlates of delayed recognition and treatment of acute type A aortic dissection.  Circulation. 2011;124(18):1911-1918.PubMedGoogle ScholarCrossref
28.
Vagnarelli  F, Corsini  A, Bugani  G,  et al.  Troponin T elevation in acute aortic syndromes [published online June 8, 2015].  Eur Heart J Acute Cardiovasc Care. doi:10.1177/2048872615590146.PubMedGoogle Scholar
29.
Moore  AG, Eagle  KA, Bruckman  D,  et al.  Choice of computed tomography, transesophageal echocardiography, magnetic resonance imaging, and aortography in acute aortic dissection.  Am J Cardiol. 2002;89(10):1235-1238.PubMedGoogle ScholarCrossref
30.
Panting  JR, Norell  MS, Baker  C, Nicholson  AA.  Feasibility, accuracy and safety of magnetic resonance imaging in acute aortic dissection.  Clin Radiol. 1995;50(7):455-458.PubMedGoogle ScholarCrossref
31.
Sommer  T, Fehske  W, Holzknecht  N,  et al.  Aortic dissection: a comparative study of diagnosis with spiral CT, multiplanar transesophageal echocardiography, and MR imaging.  Radiology. 1996;199(2):347-352.PubMedGoogle ScholarCrossref
32.
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(2):202-206.PubMedGoogle ScholarCrossref
33.
Kuehl  H, Eggebrecht  H, Boes  T,  et al.  Detection of inflammation in patients with acute aortic syndrome.  Heart. 2008;94(11):1472-1477.PubMedGoogle ScholarCrossref
34.
Di Cesare  E, Giordano  AV, Cerone  G, De Remigis  F, Deusanio  G, Masciocchi  C.  Comparative evaluation of TEE, conventional MRI and contrast-enhanced 3D breath-hold MRA in the post-operative follow-up of dissecting aneurysms.  Int J Card Imaging. 2000;16(3):135-147.PubMedGoogle ScholarCrossref
35.
Laissy  JP, Blanc  F, Soyer  P,  et al.  Thoracic aortic dissection.  Radiology. 1995;194(2):331-336.PubMedGoogle ScholarCrossref
36.
Weintraub  AR, Erbel  R, Görge  G,  et al.  Intravascular ultrasound imaging in acute aortic dissection.  J Am Coll Cardiol. 1994;24(2):495-503.PubMedGoogle ScholarCrossref
37.
Suzuki  T, Distante  A, Zizza  A,  et al.  Diagnosis of acute aortic dissection by D-dimer.  Circulation. 2009;119(20):2702-2707.PubMedGoogle ScholarCrossref
38.
Akutsu  K, Sato  N, Yamamoto  T,  et al.  A rapid bedside D-dimer assay (cardiac D-dimer) for screening of clinically suspected acute aortic dissection.  Circ J. 2005;69(4):397-403.PubMedGoogle ScholarCrossref
39.
Sbarouni  E, Georgiadou  P, Marathias  A, Geroulanos  S, Kremastinos  DT.  D-dimer and BNP levels in acute aortic dissection.  Int J Cardiol. 2007;122(2):170-172.PubMedGoogle ScholarCrossref
40.
Shao  N, Xia  S, Wang  J,  et al.  The role of D-dimers in the diagnosis of acute aortic dissection.  Mol Biol Rep. 2014;41(10):6397-6403.PubMedGoogle ScholarCrossref
41.
Wen  D, Du  X, Dong  JZ, Zhou  XL, Ma  CS.  Value of D-dimer and C reactive protein in predicting inhospital death in acute aortic dissection.  Heart. 2013;99(16):1192-1197.PubMedGoogle ScholarCrossref
42.
Huang  B, Yang  Y, Lu  H,  et al.  Impact of D-dimer levels on admission on inhospital and long-term outcome in patients with type A acute aortic dissection.  Am J Cardiol. 2015;115(11):1595-1600.PubMedGoogle ScholarCrossref
43.
Shinohara  T, Suzuki  K, Okada  M,  et al.  Soluble elastin fragments in serum are elevated in acute aortic dissection.  Arterioscler Thromb Vasc Biol. 2003;23(10):1839-1844.PubMedGoogle ScholarCrossref
44.
Giachino  F, Loiacono  M, Lucchiari  M,  et al.  Rule out of acute aortic dissection with plasma matrix metalloproteinase 8 in the emergency department.  Crit Care. 2013;17(1):R33.PubMedGoogle ScholarCrossref
45.
Suzuki  T, Katoh  H, Watanabe  M,  et al.  Novel biochemical diagnostic method for aortic dissection.  Circulation. 1996;93(6):1244-1249.PubMedGoogle ScholarCrossref
46.
Kobayashi  N, Hata  N, Kume  N,  et al.  Detection of acute aortic dissection by extremely high soluble lectin-like oxidized LDL receptor-1 (sLOX-1) and low troponin T levels in blood.  Int J Cardiol. 2013;165(3):557-559.PubMedGoogle ScholarCrossref
47.
Evangelista  A, Garcia-del-Castillo  H, Gonzalez-Alujas  T,  et al.  Diagnosis of ascending aortic dissection by transesophageal echocardiography.  J Am Coll Cardiol. 1996;27(1):102-107.PubMedGoogle ScholarCrossref
48.
Evangelista  A, Avegliano  G, Aguilar  R,  et al.  Impact of contrast-enhanced echocardiography on the diagnostic algorithm of acute aortic dissection.  Eur Heart J. 2010;31(4):472-479.PubMedGoogle ScholarCrossref
49.
Nazerian  P, Vanni  S, Castelli  M,  et al.  Diagnostic performance of emergency transthoracic focus cardiac ultrasound in suspected acute type A aortic dissection.  Intern Emerg Med. 2014;9(6):665-670.PubMedGoogle ScholarCrossref
50.
Booher  AM, Isselbacher  EM, Nienaber  CA,  et al.  The IRAD classification system for characterizing survival after aortic dissection.  American J Med.2013;126(8):730.e719-724. PubMedGoogle ScholarCrossref
51.
Conzelmann  LO, Hoffmann  I, Blettner  M,  et al.  Analysis of risk factors for neurological dysfunction in patients with acute aortic dissection type A.  Eur J Cardiothorac Surg. 2012;42(3):557-565.PubMedGoogle ScholarCrossref
52.
Muehle  A, Chou  D, Shah  A, Khoynezhad  A.  Experiences with ascending aortic endografts using FDA IDE approved devices.  J Cardiovasc Surg (Torino). 2015;56(1):11-18.PubMedGoogle Scholar
53.
Dake  MD, Kato  N, Mitchell  RS,  et al.  Endovascular stent-graft placement for the treatment of acute aortic dissection.  N Engl J Med. 1999;340(20):1546-1552.PubMedGoogle ScholarCrossref
54.
Sato  F, Kitamura  T, Kongo  M,  et al.  Newly diagnosed acute aortic dissection.  Int Heart J. 2005;46(6):1083-1098.PubMedGoogle ScholarCrossref
55.
Fattori  R, Montgomery  D, Lovato  L,  et al.  Survival after endovascular therapy in patients with type B aortic dissection.  JACC Cardiovasc Interv. 2013;6(8):876-882.PubMedGoogle ScholarCrossref
56.
Weiss  G, Tsagakis  K, Jakob  H,  et al.  The frozen elephant trunk technique for the treatment of complicated type B aortic dissection with involvement of the aortic arch.  Eur J Cardiothorac Surg. 2015;47(1):106-114.PubMedGoogle ScholarCrossref
57.
Bavaria  JE, Brinkman  WT, Hughes  GC,  et al.  Outcomes of thoracic endovascular aortic repair in acute type b aortic dissection.  Ann Thorac Surg. 2015;100(3):802-808.PubMedGoogle ScholarCrossref
58.
Li  DL, Zhang  HK, Cai  YY,  et al.  Acute type B aortic intramural hematoma.  J Endovasc Ther. 2010;17(5):617-621.PubMedGoogle ScholarCrossref
59.
Zhang  G, Feng  Q, Zheng  D,  et al.  Early aggressive medical treatment associated with selective prophylactic aortic stent-grafting for aortic intramural hematoma.  Thorac Cardiovasc Surg. 2011;59(6):342-348.PubMedGoogle ScholarCrossref
60.
Song  JK, Kim  HS, Song  JM,  et al.  Outcomes of medically treated patients with aortic intramural hematoma.  Am J Med. 2002;113(3):181-187.PubMedGoogle ScholarCrossref
61.
Zahn  R, Erbel  R, Nienaber  CA,  et al.  Endovascular aortic repair of thoracic aortic disease.  J Endovasc Ther. 2013;20(3):265-272.PubMedGoogle ScholarCrossref
62.
Nienaber  CA, Sievers  HH.  Intramural hematoma in acute aortic syndrome.  Circulation. 2002;106(3):284-285.PubMedGoogle ScholarCrossref
63.
Schlatter  T, Auriol  J, Marcheix  B,  et al.  Type B intramural hematoma of the aorta.  J Vasc Interv Radiol. 2011;22(4):533-541.PubMedGoogle ScholarCrossref
64.
Heijmen  RH, Thompson  MM, Fattori  R, Goktay  Y, Teebken  OE, Orend  KH.  Valiant thoracic stent-graft deployed with the new captivia delivery system.  J Endovasc Ther. 2012;19(2):213-225.PubMedGoogle ScholarCrossref
65.
Pepi  M, Campodonico  J, Galli  C,  et al.  Rapid diagnosis and management of thoracic aortic dissection and intramural haematoma.  Eur J Echocardiogr. 2000;1(1):72-79.PubMedGoogle ScholarCrossref
66.
von Kodolitsch  Y, Schwartz  AG, Nienaber  CA.  Clinical prediction of acute aortic dissection.  Arch Intern Med. 2000;160(19):2977-2982.PubMedGoogle ScholarCrossref
67.
Winnerkvist  A, Lockowandt  U, Rasmussen  E, Rådegran  K.  A prospective study of medically treated acute type B aortic dissection.  Eur J Vasc Endovasc Surg. 2006;32(4):349-355.PubMedGoogle ScholarCrossref
68.
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.  J Am Coll Cardiol. 2010;55(14):e27-e129.PubMedGoogle ScholarCrossref
69.
Erbel  R, Aboyans  V, Boileau  C,  et al.  2014 ESC Guidelines on the diagnosis and treatment of aortic diseases.  Eur Heart J. 2014;35(41):2873-2926.PubMedGoogle ScholarCrossref
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right

Name Your Search

Save Search
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Topics
State Requirements