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Acute Aortic Dissection and Intramural Hematoma A Systematic Review

Firas F. Mussa, MD1; Joshua D. Horton, MD2; Rameen Moridzadeh, MD3; Joseph Nicholson, PhD2; Santi Trimarchi, MD, PhD4; Kim A. Eagle, MD5
[+] Author Affiliations
1Division of Vascular Surgery, Department of Surgery, Columbia University Medical Center, New York, New York
2New York University School of Medicine, New York
3Division of Vascular Surgery, University of California-Los Angeles, Los Angeles
4Cardiovascular Surgery Department, IRCCS Policlinico San Donato University of Milan, Milan, Italy
5Division of Cardiology, Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor
JAMA. 2016;316(7):754-763. doi:10.1001/jama.2016.10026.
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Published online

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.

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Figure 1.
Anatomy of the Aorta and Pathogenesis of Acute Aortic Syndrome

FL indicates false lumen; TL, true lumen. In B, note the intimal entry tear in aortic dissection and lack of this entry tear in intramural hematoma. Penetrating atherosclerotic ulcer is characterized by significant atherosclerotic plaque, which may erode through the intima creating a communication between the aortic lumen and the media.

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Figure 2.
Clinical Imaging of Aortic Dissection and Intramural Hematoma From Different Patients

A, Type A aortic dissection, axial contrast enhanced computed tomography (CT) at the level of the pulmonary artery bifurcation (star = false lumen). B, Type B aortic dissection, axial contrast enhanced CT at the level of the carina (star = false lumen). C, Type A aortic dissection, sagittal contrast enhanced CT (arrowhead = dissection plane). D, Type B aortic dissection, sagittal contrast enhanced CT. Note the false lumen along the inner (anterior) curve of the aorta. (arrowhead = dissection plane). E, Type A intramural hematoma, coronal contrast enhanced CT (star = hematoma). F, Type B intramural hematoma, sagittal contrast enhanced CT. Arrowheads point to hematoma within the wall of the descending aorta. Note heterogeneous hyperintensity corresponding to blood.

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Figure 3.
Stanford and DeBakey Classification of Acute Aortic Syndrome

Stanford type A lesions involve the ascending aorta, whereas type B lesions are confined to the descending aorta. The DeBakey system accounts for pathology affecting both the ascending and descending aorta (I), only the ascending segment (II), or only the descending portion (III).

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