Acute aortic syndrome
2010; Elsevier BV; Volume: 140; Issue: 6 Linguagem: Inglês
10.1016/j.jtcvs.2010.07.062
ISSN1097-685X
AutoresSteven Lansman, Paul Saunders, Ramin Malekan, David Spielvogel,
Tópico(s)Aortic aneurysm repair treatments
ResumoThe term acute aortic syndrome refers to a heterogeneous group of conditions that cause a common set of signs and symptoms, the foremost of which is aortic pain. Various pathologic entities may give rise to this syndrome, but the topic has come to focus on penetrating aortic ulcer and intramural hematoma and their relation to aortic dissection. Penetrating aortic ulcer is a focal atherosclerotic plaque that corrodes a variable depth through the intima into the media. Intramural hematoma is a blood collection within the aortic wall not freely communicating with the aortic lumen, with restricted flow. It may represent a subcategory of aortic dissection that manifests different behavior by virtue of limited flow in the false lumen. This article reviews the current literature regarding acute aortic syndrome, focusing on management options. The term acute aortic syndrome refers to a heterogeneous group of conditions that cause a common set of signs and symptoms, the foremost of which is aortic pain. Various pathologic entities may give rise to this syndrome, but the topic has come to focus on penetrating aortic ulcer and intramural hematoma and their relation to aortic dissection. Penetrating aortic ulcer is a focal atherosclerotic plaque that corrodes a variable depth through the intima into the media. Intramural hematoma is a blood collection within the aortic wall not freely communicating with the aortic lumen, with restricted flow. It may represent a subcategory of aortic dissection that manifests different behavior by virtue of limited flow in the false lumen. This article reviews the current literature regarding acute aortic syndrome, focusing on management options. The term acute aortic syndrome (AAS), coined by Vilacosta and associates1Vilacosta I. San Román J.A. Aragoncillo P. Ferreirós J. Mendez R. Graupner C. et al.Penetrating atherosclerotic aortic ulcer: documentation by transesophageal echocardiography.J Am Coll Cardiol. 1998; 32: 83-89Abstract Full Text Full Text PDF PubMed Scopus (120) Google Scholar, 2Vilacosta I. San Román J.A. Acute aortic syndrome.Heart. 2001; 85: 365-368Crossref PubMed Scopus (239) Google Scholar in 1998, refers to a heterogeneous group of conditions that cause a common set of signs and symptoms, the foremost of which is aortic pain. The pain is acute, severely intense—often maximally so at its outset—and may be described as tearing, ripping, migrating, or pulsating. Hirst, Johns, and Kime3Hirst Jr., A.E. Johns Jr., V.J. Kime Jr., S.W. Dissecting aneurysm of the aorta: a review of 505 cases.Medicine (Baltimore). 1958; 37: 217-279Crossref PubMed Scopus (1071) Google Scholar state: “The patient will frequently volunteer the information that it feels as if ‘something has broken loose’ in the chest.” Various diseases may cause this striking presentation, including trauma, pseudoaneurysm, and ruptured atherosclerotic aneurysm, but the term has come to subsume penetrating aortic ulcer (PAU), intramural hematoma (IMH), and aortic dissection (AD). One might ask whether a special category is needed for these entities, but the term does seem to serve a function; like “acute coronary syndrome” and “acute abdomen,” it isolates a set of pathologic conditions that may be unrelated etiologically but nonetheless have a similar presentation and require emergency attention. PAU, initially described by Shennan4Shennan T. Dissecting aneurysms. Medical Research Council, Special Report Series, No 193. 1934.Google Scholar in 1934, is a focal atherosclerotic plaque that corrodes a variable depth through the internal elastic lamina into the media. It may form a pseudoaneurysm or may rupture into the media, forming an IMH in the media5Macura K.J. Corl F.M. Fishman E.K. Bluemke D.A. Pathogenesis in acute aortic syndromes: aortic dissection, intramural hematoma, and penetrating atherosclerotic aortic ulcer.AJR Am J Roentgenol. 2003; 181: 309-316Crossref PubMed Scopus (133) Google Scholar, 6Vilacosta I. Aragoncillo P. Cañadas V. San Román J.A. Ferreirós J. Rodríguez E. Acute aortic syndrome: a new look at an old conundrum.Heart. 2009; 95: 1130-1139PubMed Google Scholar or between the media and adventitia.4Shennan T. Dissecting aneurysms. Medical Research Council, Special Report Series, No 193. 1934.Google Scholar, 6Vilacosta I. Aragoncillo P. Cañadas V. San Román J.A. Ferreirós J. Rodríguez E. Acute aortic syndrome: a new look at an old conundrum.Heart. 2009; 95: 1130-1139PubMed Google Scholar, 7Demers P. Miller D.C. Mitchell R.S. Kee S.T. Chagonjian L. Dake M.D. Stent-graft repair of penetrating atherosclerotic ulcers in the descending thoracic aorta: mid-term results.Ann Thorac Surg. 2004; 77: 81-86Abstract Full Text Full Text PDF PubMed Scopus (111) Google Scholar The hematoma may propagate locally or, rarely, may give rise to classic AD. PAUs tend to occur in older men.8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar, 9Troxler M. Mavor A.I.D. Homer-Vanniasinkam S. Penetrating atherosclerotic ulcers of the aorta.Br J Surg. 2001; 88: 1169-1177Crossref PubMed Scopus (85) Google Scholar, 10Coady M.A. Rizzo J.A. Elefteriades J.A. Pathologic variants of thoracic aortic dissections. penetrating atherosclerotic ulcers and intramural hematomas.Cardiol Clin. 1999; 17: 637-657Abstract Full Text Full Text PDF PubMed Scopus (216) Google Scholar The lesions may be single or multiple (31.6% in the series reported by Botta and associates11Botta L. Buttazzi K. Russo V. Parlapiano M. Gostoli V. Di Bartolomeo R. et al.Endovascular repair for penetrating atherosclerotic ulcers of the descending thoracic aorta: early and mid-term results.Ann Thorac Surg. 2008; 85: 987-992Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar), are predominantly situated in the descending aorta,9Troxler M. Mavor A.I.D. Homer-Vanniasinkam S. Penetrating atherosclerotic ulcers of the aorta.Br J Surg. 2001; 88: 1169-1177Crossref PubMed Scopus (85) Google Scholar, 12Gottardi R. Zimpfer D. Funovics M. Schoder M. Lammer J. Wolner E. et al.Mid-term results after endovascular stent-graft placement due to penetrating atherosclerotic ulcers of the thoracic aorta.Eur J Cardiothorac Surg. 2008; 33: 1019-1024Crossref PubMed Scopus (28) Google Scholar but less frequently occur in the arch1Vilacosta I. San Román J.A. Aragoncillo P. Ferreirós J. Mendez R. Graupner C. et al.Penetrating atherosclerotic aortic ulcer: documentation by transesophageal echocardiography.J Am Coll Cardiol. 1998; 32: 83-89Abstract Full Text Full Text PDF PubMed Scopus (120) Google Scholar, 13Stanson A.W. Kazmier F. Hollier L. Edwards W. Pairolero P. Sheedy P. et al.Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations.Ann Vasc Surg. 1986; 1: 15-23Abstract Full Text PDF PubMed Scopus (450) Google Scholar, 14Coady M.A. Rizzo J.A. Hammond G.L. Pierce J.G. Kopf G.S. Elefteriades J.A. Penetrating ulcer of the thoracic aorta: What is it? How do we recognize it? How do we manage it?.J Vasc Surg. 1998; 27: 1006-1016Abstract Full Text Full Text PDF PubMed Scopus (278) Google Scholar, 15Quint L.E. Williams D.M. Francis I.R. Monaghan H.M. Sonnad S.S. Patel S. et al.Ulcerlike lesions of the aorta: imaging features and natural history.Radiology. 2001; 218: 719-723Crossref PubMed Scopus (137) Google Scholar or abdominal15Quint L.E. Williams D.M. Francis I.R. Monaghan H.M. Sonnad S.S. Patel S. et al.Ulcerlike lesions of the aorta: imaging features and natural history.Radiology. 2001; 218: 719-723Crossref PubMed Scopus (137) Google Scholar, 16Harris J.A. Bis K.G. Glover J.L. Bendick P.J. Shetty A. Brown O.W. Penetrating atherosclerotic ulcers of the aorta.J Vasc Surg. 1994; 19 (discussion 8–9): 90-98Abstract Full Text Full Text PDF PubMed Scopus (208) Google Scholar segments, and rarely in the ascending aorta.1Vilacosta I. San Román J.A. Aragoncillo P. Ferreirós J. Mendez R. Graupner C. et al.Penetrating atherosclerotic aortic ulcer: documentation by transesophageal echocardiography.J Am Coll Cardiol. 1998; 32: 83-89Abstract Full Text Full Text PDF PubMed Scopus (120) Google Scholar, 8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar, 13Stanson A.W. Kazmier F. Hollier L. Edwards W. Pairolero P. Sheedy P. et al.Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations.Ann Vasc Surg. 1986; 1: 15-23Abstract Full Text PDF PubMed Scopus (450) Google Scholar, 14Coady M.A. Rizzo J.A. Hammond G.L. Pierce J.G. Kopf G.S. Elefteriades J.A. Penetrating ulcer of the thoracic aorta: What is it? How do we recognize it? How do we manage it?.J Vasc Surg. 1998; 27: 1006-1016Abstract Full Text Full Text PDF PubMed Scopus (278) Google Scholar, 15Quint L.E. Williams D.M. Francis I.R. Monaghan H.M. Sonnad S.S. Patel S. et al.Ulcerlike lesions of the aorta: imaging features and natural history.Radiology. 2001; 218: 719-723Crossref PubMed Scopus (137) Google Scholar In the Mayo Clinic series,8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar patient characteristics included hypertension (92%), tobacco use (77%), and coronary artery disease (46%); 75% were symptomatic, usually with back pain, and 30% had pleural effusions. Chronic obstructive pulmonary disease is common (24% in the Mayo series8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar; 68.4% in Botta and associates' series11Botta L. Buttazzi K. Russo V. Parlapiano M. Gostoli V. Di Bartolomeo R. et al.Endovascular repair for penetrating atherosclerotic ulcers of the descending thoracic aorta: early and mid-term results.Ann Thorac Surg. 2008; 85: 987-992Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar), as are concurrent aneurysms, particularly in the abdomen (42.1% in the Yale series10Coady M.A. Rizzo J.A. Elefteriades J.A. Pathologic variants of thoracic aortic dissections. penetrating atherosclerotic ulcers and intramural hematomas.Cardiol Clin. 1999; 17: 637-657Abstract Full Text Full Text PDF PubMed Scopus (216) Google Scholar; 61% in the Mayo series8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar). PAU is best diagnosed by contrast-enhanced computed tomographic (CT) scan or magnetic resonance imaging, appearing as a focal, contrast-filled outpouching with irregular margins, and extending beyond the expected aortic wall boundaries. It generally occurs in the presence of severe atheromatous disease.5Macura K.J. Corl F.M. Fishman E.K. Bluemke D.A. Pathogenesis in acute aortic syndromes: aortic dissection, intramural hematoma, and penetrating atherosclerotic aortic ulcer.AJR Am J Roentgenol. 2003; 181: 309-316Crossref PubMed Scopus (133) Google Scholar, 17Litmanovich D. Bankier A.A. Cantin L. Raptopoulos V. Boiselle P.M. CT and MRI in diseases of the aorta.AJR Am J Roentgenol. 2009; 193: 928-940Crossref PubMed Scopus (102) Google Scholar, 18Yoo S.M. Lee H.Y. White C.S. MDCT evaluation of acute aortic syndrome.Radiol Clin North Am. 2010; 48: 67-83Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar The incidence of PAU in AASs ranges from 2.3% to 11%.19Brinster D.R. Endovascular repair of the descending thoracic aorta for penetrating atherosclerotic ulcer disease.J Card Surg. 2009; 24: 203-208Crossref PubMed Scopus (27) Google Scholar In Hirst and Barbour's comprehensive autopsy series,20Hirst Jr., A.E. Barbour B.H. Dissecting aneurysm with hemopericardium: report of a case with healing.N Engl J Med. 1958; 258: 116-120Crossref PubMed Scopus (17) Google Scholar 4.6% (18/398) of dissections originated from PAUs. The prevalence of PAU is unknown but may be less than once suspected, inasmuch as the widespread use of coronary CT scans has not uncovered large numbers of incidental cases.21MacHaalany J. Yam Y. Ruddy T.D. Abraham A. Chen L. Beanlands R.S. et al.Potential clinical and economic consequences of noncardiac incidental findings on cardiac computed tomography.J Am Coll Cardiol. 2009; 54: 1533-1541Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar One recent study revealed only 2 PAUs in 966 cardiac CT coronary angiograms,21MacHaalany J. Yam Y. Ruddy T.D. Abraham A. Chen L. Beanlands R.S. et al.Potential clinical and economic consequences of noncardiac incidental findings on cardiac computed tomography.J Am Coll Cardiol. 2009; 54: 1533-1541Abstract Full Text Full Text PDF PubMed Scopus (128) Google Scholar and another found but 1 PAU in 395 CTs obtained for suspected acute coronary syndrome.22Lehman S.J. Abbara S. Cury R.C. Nagurney J.T. Hsu J. Goela A. et al.Significance of cardiac computed tomography incidental findings in acute chest pain.Am J Med. 2009; 122: 543-549Abstract Full Text Full Text PDF PubMed Scopus (76) Google Scholar Conflicting reports about the natural history of PAU have led to opposing management strategies. Concerned with the “potentially progressive and serious nature” of PAU, Stanson and his colleagues13Stanson A.W. Kazmier F. Hollier L. Edwards W. Pairolero P. Sheedy P. et al.Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations.Ann Vasc Surg. 1986; 1: 15-23Abstract Full Text PDF PubMed Scopus (450) Google Scholar at the Mayo Clinic in 1986 initially adopted an aggressive stance, but their approach changed as conservative therapy proved successful23Sundt T.M. Intramural hematoma and penetrating atherosclerotic ulcer of the aorta.Ann Thorac Surg. 2007; 83: S835-S841Abstract Full Text Full Text PDF PubMed Scopus (101) Google Scholar: in 105 cases, 30-day mortality for medical and surgical treatment was 4% and 21% (P < .05). Moreover, in 89%, mean IMH thickness decreased by 1 month, completely resolving by 1 year in 85%, and only 6 patients needed late surgery for aortic expansion.8Cho K.R. Stanson A.W. Potter D.D. Cherry K.J. Schaff H.V. Sundt III, T.M. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch.J Thorac Cardiovasc Surg. 2004; 127: 1393-1401Abstract Full Text Full Text PDF PubMed Scopus (178) Google Scholar Hussain and associates24Hussain S. Glover J.L. Bree R. Bendick P.J. Penetrating atherosclerotic ulcers of the thoracic aorta.J Vasc Surg. 1989; 9: 710-717PubMed Scopus (91) Google Scholar also reported a benign course for PAU in 5 patients: although 4 were symptomatic, all survived without surgery, with complete resolution in 4. A CT study evaluating 56 PAUs in 38 patients did not reveal any feature predictive of lesion progression, although pleural effusion did correlate with clinical instability.15Quint L.E. Williams D.M. Francis I.R. Monaghan H.M. Sonnad S.S. Patel S. et al.Ulcerlike lesions of the aorta: imaging features and natural history.Radiology. 2001; 218: 719-723Crossref PubMed Scopus (137) Google Scholar Only 30% progressed, manifesting a mild–moderate increase in lesion size, aortic diameter, or both, whereas 76% were asymptomatic, with clinically stable lesions. Thus, on the basis of satisfactory results with a conservative approach, a number of authors have recommended initial supportive therapy for PAU, with surgery reserved to treat or prevent rupture, as indicated by persistent pain or enlarging PAU or aortic size. PAU proved less benign in other series. For example, Harris and colleagues,16Harris J.A. Bis K.G. Glover J.L. Bendick P.J. Shetty A. Brown O.W. Penetrating atherosclerotic ulcers of the aorta.J Vasc Surg. 1994; 19 (discussion 8–9): 90-98Abstract Full Text Full Text PDF PubMed Scopus (208) Google Scholar monitoring 17 ulcers in 10 patients, reported distal embolization in 1 and progression to fusiform or saccular aneurysm formation in 12% and 29%. The Yale group reported that 40% of 15 medically managed patients needed emergency surgery for rupture.14Coady M.A. Rizzo J.A. Hammond G.L. Pierce J.G. Kopf G.S. Elefteriades J.A. Penetrating ulcer of the thoracic aorta: What is it? How do we recognize it? How do we manage it?.J Vasc Surg. 1998; 27: 1006-1016Abstract Full Text Full Text PDF PubMed Scopus (278) Google Scholar An updated review of their series, showing rupture in 38%, surgical intervention in 65%, and hospital mortality in 15%, reinforced their advocacy of aggressive management for PAU.25Tittle S.L. Lynch R.J. Cole P.E. Singh H.S. Rizzo J.A. Kopf G.S. et al.Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta.J Thorac Cardiovasc Surg. 2002; 123: 1051-1059Abstract Full Text Full Text PDF PubMed Scopus (207) Google Scholar The Stanford group also advocated an aggressive approach on the basis of their experience with 65 IMH cases, wherein progression with and without PAU was 48% and 8% and medical management resulted in 10% mortality.26Ganaha F. Miller D.C. Sugimoto K. Do Y.S. Minamiguchi H. Saito H. et al.Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis.Circulation. 2002; 106: 342-348Crossref PubMed Scopus (377) Google Scholar Predictors of progression included sustained or recurrent pain (P < .0001), increasing pleural effusion (P = .0003), and both the maximum PAU diameter (P = .004) and maximum PAU depth (P = .003), with recommended threshold values of 20 mm for PAU diameter and 10 mm for PAU depth. Whether PAUs are initially treated more or less aggressively, both camps advocate intervention for clear-cut indications of pending or actual rupture, and over the past decade endovascular treatment has emerged as an attractive option. PAU comprised 7.1% of a series of 113 consecutive patients with acute disease in the Talent Thoracic Retrospective Registry.27Kaya A. Heijmen R.H. Rousseau H. Nienaber C.A. Ehrlich M. Amabile P. et al.Emergency treatment of the thoracic aorta: results in 113 consecutive acute patients (the Talent Thoracic Retrospective Registry).Eur J Cardiothorac Surg. 2009; 35: 276-281Crossref PubMed Scopus (31) Google Scholar One might think endograft therapy an attractive setting for PAU, inasmuch as most are isolated and localized in a relatively normal-sized aorta, but there are a number of caveats. First, PAU occurs in an older group having significant comorbidities, often including chronic obstructive pulmonary disease, coronary artery disease, and renal insufficiency. Also, apparently isolated PAUs often arise in a diffuse atherosclerotic setting, which may increase the incidence of type I endoleaks—18% in a recent series.11Botta L. Buttazzi K. Russo V. Parlapiano M. Gostoli V. Di Bartolomeo R. et al.Endovascular repair for penetrating atherosclerotic ulcers of the descending thoracic aorta: early and mid-term results.Ann Thorac Surg. 2008; 85: 987-992Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar Luminal irregularity may therefore necessitate more extensive endografting than anticipated and—as opposed to atherosclerotic aneurysms, where intercostal branches are often thrombosed—intercostal vessels may be patent in PAU, increasing the risk of paraplegia by endograft occlusion.12Gottardi R. Zimpfer D. Funovics M. Schoder M. Lammer J. Wolner E. et al.Mid-term results after endovascular stent-graft placement due to penetrating atherosclerotic ulcers of the thoracic aorta.Eur J Cardiothorac Surg. 2008; 33: 1019-1024Crossref PubMed Scopus (28) Google Scholar Nonetheless, endograft treatment of PAU has yielded excellent perioperative results for these high-risk patients. The Stanford group reported 94% primary success in 26 patients, with 12% 30-day mortality and 0% paraplegia, although half of the PAUs were deemed inoperable and 6 were ruptured7Demers P. Miller D.C. Mitchell R.S. Kee S.T. Chagonjian L. Dake M.D. Stent-graft repair of penetrating atherosclerotic ulcers in the descending thoracic aorta: mid-term results.Ann Thorac Surg. 2004; 77: 81-86Abstract Full Text Full Text PDF PubMed Scopus (111) Google Scholar; however, underscoring the significance of comorbidities, the 5-year survival was only 70%. Similarly, Botta and coworkers,11Botta L. Buttazzi K. Russo V. Parlapiano M. Gostoli V. Di Bartolomeo R. et al.Endovascular repair for penetrating atherosclerotic ulcers of the descending thoracic aorta: early and mid-term results.Ann Thorac Surg. 2008; 85: 987-992Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar with 0% intraoperative mortality in 19 cases, and Eggebrecht and colleagues,28Eggebrecht H. Herold U. Schmermund A. Lind A.Y. Kuhnt O. Martini S. et al.Endovascular stent-graft treatment of penetrating aortic ulcer: results over a median follow-up of 27 months.Am Heart J. 2006; 151: 530-536Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar with 0% 30-day mortality in 22 cases, reported 5-year survivals of 66.7% and 62%. Criteria for endograft placement in the acute setting, advanced by Botta's group,11Botta L. Buttazzi K. Russo V. Parlapiano M. Gostoli V. Di Bartolomeo R. et al.Endovascular repair for penetrating atherosclerotic ulcers of the descending thoracic aorta: early and mid-term results.Ann Thorac Surg. 2008; 85: 987-992Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar include pain and rupture; in chronic cases, indications include recurrent pain, aortic diameter greater than 55 mm, and increase in size greater than 10 mm per year. IMH, first described by Krukenberg29Krukenberg E. Beiträge zur Frage des Aneurysma dissecans.Beitr Pathol Anat Allg Pathol. 1920; 67: 329-351Google Scholar in 1920, has been described as “dissection without intimal tear.” Histologically, the hematoma generally extends within the media, but it may be subadventitial.6Vilacosta I. Aragoncillo P. Cañadas V. San Román J.A. Ferreirós J. Rodríguez E. Acute aortic syndrome: a new look at an old conundrum.Heart. 2009; 95: 1130-1139PubMed Google Scholar On diagnostic imaging, IMH appears as a smooth, crescentic, or circular thickening greater than 5 to 7 mm. With expansion, the hematoma may encroach on the aortic lumen and, if intimal calcium is present, displace it centrally. Transthoracic or transesophageal echocardiography may demonstrate IMH, but multidetector CT is the best diagnostic modality, inasmuch as it can image the entire aorta and branch vessels.18Yoo S.M. Lee H.Y. White C.S. MDCT evaluation of acute aortic syndrome.Radiol Clin North Am. 2010; 48: 67-83Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar IMH comprises 10% to 30% of AAS.6Vilacosta I. Aragoncillo P. Cañadas V. San Román J.A. Ferreirós J. Rodríguez E. Acute aortic syndrome: a new look at an old conundrum.Heart. 2009; 95: 1130-1139PubMed Google Scholar The patients tend to be older,10Coady M.A. Rizzo J.A. Elefteriades J.A. Pathologic variants of thoracic aortic dissections. penetrating atherosclerotic ulcers and intramural hematomas.Cardiol Clin. 1999; 17: 637-657Abstract Full Text Full Text PDF PubMed Scopus (216) Google Scholar and most are hypertensive.10Coady M.A. Rizzo J.A. Elefteriades J.A. Pathologic variants of thoracic aortic dissections. penetrating atherosclerotic ulcers and intramural hematomas.Cardiol Clin. 1999; 17: 637-657Abstract Full Text Full Text PDF PubMed Scopus (216) Google Scholar, 30Robbins R. McManus R. Mitchell R. Latter D. Moon M. 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O'Gara P.T. Fattori R. Cooper J.V. et al.Acute intramural hematoma of the aorta: a mystery in evolution.Circulation. 2005; 111: 1063-1070Crossref PubMed Scopus (361) Google Scholar Type A IMH is often complicated by pericardial effusion (47%34Kan C.-B. Chang R.-Y. Chang J- P. Optimal initial treatment and clinical outcome of type A aortic intramural hematoma: a clinical review.Eur J Cardiothorac Surg. 2008; 33: 1002-1006Crossref PubMed Scopus (41) Google Scholar–68.3%33Song J.-K. Yim J.H. Ahn J.-M. Kim D.-H. Kang J.-W. Lee T.Y. et al.Outcomes of patients with acute type A aortic intramural hematoma.Circulation. 2009; 120: 2046-2052Crossref PubMed Scopus (127) Google Scholar), pleural effusion (49%33Song J.-K. Yim J.H. Ahn J.-M. Kim D.-H. Kang J.-W. Lee T.Y. et al.Outcomes of patients with acute type A aortic intramural hematoma.Circulation. 2009; 120: 2046-2052Crossref PubMed Scopus (127) Google Scholar) and aortic insufficiency (11.9%33Song J.-K. Yim J.H. Ahn J.-M. Kim D.-H. Kang J.-W. Lee T.Y. et al.Outcomes of patients with acute type A aortic intramural hematoma.Circulation. 2009; 120: 2046-2052Crossref PubMed Scopus (127) Google Scholar–21%34Kan C.-B. Chang R.-Y. Chang J- P. Optimal initial treatment and clinical outcome of type A aortic intramural hematoma: a clinical review.Eur J Cardiothorac Surg. 2008; 33: 1002-1006Crossref PubMed Scopus (41) Google Scholar). Interestingly, Marfan syndrome (1%)33Song J.-K. Yim J.H. Ahn J.-M. Kim D.-H. Kang J.-W. Lee T.Y. et al.Outcomes of patients with acute type A aortic intramural hematoma.Circulation. 2009; 120: 2046-2052Crossref PubMed Scopus (127) Google Scholar and bicuspid aortic valve were uncommon in many series. It is commonly held that AD arises from an intimal tear, whereas IMH arises from “rhexis” of the vasa vasorum, without an intimal tear.5Macura K.J. Corl F.M. Fishman E.K. Bluemke D.A. Pathogenesis in acute aortic syndromes: aortic dissection, intramural hematoma, and penetrating atherosclerotic aortic ulcer.AJR Am J Roentgenol. 2003; 181: 309-316Crossref PubMed Scopus (133) Google Scholar However, the issue has long been debated, and many hold that a single mechanism gives rise to both entities; some believe that IMH represents AD with a thrombosed false lumen, whereas others believe IMH represents intramedial hemorrhage, without rupture into the true lumen. Hirst and associates,3Hirst Jr., A.E. Johns Jr., V.J. Kime Jr., S.W. Dissecting aneurysm of the aorta: a review of 505 cases.Medicine (Baltimore). 1958; 37: 217-279Crossref PubMed Scopus (1071) Google Scholar who found 21 (4%) IMHs in their 1958 review of 504 AD cases, supported the latter theory, noting, “The concept of initiation of aortic dissection by rupture of the vasa vasorum is not new, having been favored by Krukenberg in 1920.” As evidence, they cited necropsy reports of hematomas without tears (and tears without hematomas) going back to 1936. IMH may also arise from trauma,35Vilacosta I. San Román J.A. Ferreirós J. Aragoncillo P. Méndez R. Castillo J.A. et al.Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection.Am Heart J. 1997; 134: 495-507Abstract Full Text Full Text PDF PubMed Scopus (158) Google Scholar or rupture of a PAU into the media.4Shennan T. Dissecting aneurysms. Medical Research Council, Special Report Series, No 193. 1934.Google Scholar Krukenberg26Ganaha F. Miller D.C. Sugimoto K. Do Y.S. Minamiguchi H. Saito H. et al.Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis.Circulation. 2002; 106: 342-348Crossref PubMed Scopus (377) Google Scholar, 29Krukenberg E. Beiträge zur Frage des Aneurysma dissecans.Beitr Pathol Anat Allg Pathol. 1920; 67: 329-351Google Scholar described IMH as “dissection without tear,” but a number of studies emphasize the prevalence of tears in supposed cases of IMH. For example, Park and colleagues36Park K.-H. Lim C. Choi J.H. Sung K. Kim K. Lee Y.T. et al.Prevalence of aortic intimal defect in surgically treated acute type A intramural hematoma.Ann Thorac Surg. 2008; 86: 1494-1500Abstract Full Text Full Text PDF PubMed Scopus (68) Google Scholar found intimal tears on retrospectively reviewed preoperative CTs in 48.6% of 37 cases and in 73% at surgery; in 67%, the defects were located in the arch or distal ascending aorta, emphasizing the need to inspect the aorta during circulatory arrest.37Lansman S.L. McCullough J.N. Nguyen K.H. Spielvogel D. Klein J.J. Galla J.D. et al.Subtypes of acute aortic dissection.Ann Thorac Surg. 1999; 67: 1975-1978Abstract Full Text Full Text PDF PubMed Scopus (97) Google Scholar Similarly, Svensson and coworkers38Svensson L.G. Labib S.B. Eisenhauer A.C. Butterly J.R. Int
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