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82 Raghupathy and Eagle
interventions such as aortic stenting and fenestration, even during the same pro-
cedure. Invasive intravascular ultrasound may also be used during the procedure,
improving the sensitivity of aortography alone to nearly 100%. Still, aortography
is best reserved for use as a third or fourth line test (Figs. 4C and D), or in appro-
priately selected cases that require endovascular repair (25,37,39,45).
Magnetic Resonance Imaging
Although it offers superior image resolution and perhaps the highest sensitivity
and specificity for diagnosing acute aortic conditions, MRI is not often selected as
the initial imaging study. In IRAD, MRI was a selected imaging modality in 19%
of patients, but was chosen as the initial study in only 1% (Fig. 4A) (39,41,42–46).
Practical issues such as the inability to monitor an unstable patient, limited avail-
ability of equipment, and the incompatibility with implanted devices or prostheses
may in part explain the underutilization of this modality. When it is used, however,
MRI can visualize even very small tears as well as precisely characterize branch
vessel involvement. It is ideally applied to the stable patient for whom a second
imaging study is required (5,13,39).
PUTTING IT ALL TOGETHER
With the inherent diagnostic challenges and extreme urgency surrounding the
patient presenting with acute aortic condition, a decisive and efficient approach
should be consistently applied using a high index of suspicion (Fig. 10). Aortic
dissection or its variants must be considered in any patient with thoracic pain,
particularly of a sudden onset, or in any patient with unexplained heart failure,
syncope, or neurological deficit. Once the diagnosis is suspected, the emergency
Figure 9 (A and B) Transesophageal echocardiogram performed in patient presenting
with acute Type A aortic dissection. (A) Short axis view at approximately 60 degrees
demonstrating a mobile dissection flap in the ascending aorta. (B) Flow Doppler showing
flow pattern in true and false lumen. Source: Courtesy of Dr. William F. Armstrong,
University of Michigan, Ann Arbor.
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Putting It All Together: Symptoms, Signs, and Images 83
room physician should promptly alert surgical and cardiovascular services to the
potential scenario while confirmatory testing is pursued. In general, a relatively
stable patient may undergo whichever imaging test is most readily available,
keeping in mind that serious complications may develop quickly and continuous
monitoring for change in status is necessary. In the hemodynamically unstable
patient, TEE is the preferred initial test, provided that qualified personnel are
readily available to rapidly perform and interpret the test. If emergency TEE is
not an option, CT, aortography, or MRI may be pursued to confirm the diagnosis.
If imaging tests are unavailable, the unstable patient should be emergently
transferred to a tertiary referral center, preferably one with aortic expertise, for
definitive diagnosis and therapy.
Figure 10 Suggested approach to diagnosing acute aortic conditions. Abbreviations: CT,
computed tomography; ECG, electrocardiogram; MRI, magnetic resonance imaging; TEE,
transesophageal echocardiograph.
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84 Raghupathy and Eagle
EDITOR’S COMMENT
Aortic dissection can be an easy diagnosis, in the presence of typical symptoms,
signs, and radiographic findings. As aortic specialists, we see patients already
screened and imaged; we see them through the always accurate “retrospecto-
scope.” Our plea to those on the front lines, especially in the Emergency Department,
is that aortic dissection be part of the mindset, in the differential diagnosis of a
wide variety of presentations. If aortic dissection is even remotely entertained,
please image the patient. Once a CT scan, TEE, or MRI scan has been performed,
the correct diagnosis sis all but assured. We respect our colleagues in the Emergency
Department for the great job they do “sifting out” the few aortic dissection cases
from the hundreds of patients they see with chest or abdominal symptoms. (See also
Section 5: Litigation in Aortic Disease.)
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hematoma of the aorta. J Thorac Cardiovasc Surg 2002; 123(6):1051–1059.
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Putting It All Together: Symptoms, Signs, and Images 85
11. Januzzi JL, Sabatine MS, Eagle KA, et al. Iatrogenic aortic dissection. Am J Cardiol
2002; 89(5):623–626.
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tion, management, and outcomes of acute type A aortic dissection in patients
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eds. Practical Cardiology. Philadelphia: Lippincot Williams & Wilkins, 2003:459–467.
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hematoma of the ascending aorta. Ann Thorac Surg 1999; 68(5):1855–1856.
15. Coady MA, Rizzo JA, Elefteriades JA. Pathologic variants of thoracic aortic dissec-
tions: penetrating atherosclerotic ulcers and intramural hematomas. Cardiol Clin 1999;
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aorta: a mystery in evolution. Circulation 2005; 111(8):1063–1070.
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toma in patients with acute aortic dissection (from the international registry of acute
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19. Nienaber CA, Sievers H.-H. Intramural hematoma in acute aortic syndrome: more
than one variant of dissection? Circulation 2002; 106(3):284–285.
20. Nienaber CA, Von Kodolitsch Y, Petersen B, et al. Intramural hemorrhage of the tho-
racic aorta : diagnostic and therapeutic implications. Circulation 1995; 92(6):
1465–1472.
21. Von Kodolitsch Y, Csosz SK, Koschyk DH, et al. Intramural hematoma of the aorta:
predictors of progression to dissection and rupture. Circulation 2003; 107(8):
1158–1163.
22. Eggebrecht H, Naber CK, Bruch C, et al. Value of plasma fibrin D-dimers for detec-
tion of acute aortic dissection. J Am Coll Cardiol 2004; 44(4):804–809.
23. Suzuki T, Mehta RH, Ince H, et al. Clinical profiles and outcomes of acute type b
aortic dissection in the current era: lessons from the international registry of aortic
dissection (IRAD). Circulation 2003; 108(suppl 1):II-312–317.
24. Nallamothu BK, Mehta RH, Saint S, et al. Syncope in acute aortic dissection:
diagnostic, prognostic, and clinical implications. Am J Med 2002; 113(6):
468–471.
25. Sullivan PR, Wolfson AB, Leckey RD, et al. Diagnosis of acute thoracic aortic
dissection in the emergency department. Am J Emerg Med 2000; 18(1):46–50.
26. Park SW, Hutchison S, Mehta RH, et al. Association of painless acute aortic
dissection with increased mortality. Mayo Clin Proc 2004; 79(10):1252–1257.
27. Upchurch GR Jr, Nienaber C, Fattori R, et al. Acute aortic dissection presenting with
primarily abdominal pain: a rare manifestation of a deadly disease. Ann Vasc Surg
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28. Von Kodolitsch Y, Schwartz AG, Nienaber CA. Clinical prediction of acute aortic
dissection. Arch Int Med 2000; 160(19):2977–2982.
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86 Raghupathy and Eagle
30. Erbel R, Alfonso F, Boileau C, et al. Diagnosis and management of aortic dissection:
task force on aortic dissection, european society of cardiology. Eur Heart J 2001;
22(18):1642–1681.
31. Bossone E, Rampoldi V, Nienaber CA, et al. Usefulness of pulse deficit to predict
in-hospital complications and mortality in patients with acute type A aortic dissection.
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32. Erbel R, Engberding R, Daniel W, et al. Echocardiography in diagnosis of aortic
dissection. Lancet 1989; 1(8636):457–461.
33. Isselbacher E, Cigarroa J, Eagle K. Cardiac tamponade complicating proximal aortic
dissection: is pericardiocentesis harmful? Circulation 1994; 90(5):2375–2378.
34. Nallamothu BK, Eagle KA. When zebras run with horses: the diagnostic dilemma of
acute aortic dissection complicated by myocardial infarction. J Interv Cardiol 2002;
15(4):297–299.
35. Kamp TJ, Goldschmidt-Clermont PJ, Brinker JA, et al. Myocardial infarction, aortic
dissection, and thrombolytic therapy. Am Heart J 1994; 128(6 Pt 1):1234–1237.
36. Von Kodolitsch Y, Nienaber CA, Dieckmann C, et al. Chest radiography for the
diagnosis of acute aortic syndrome. Am J Med 2004; 116(2):73–77.
37. Cigarroa JE, Isselbacher EM, Desanctis RW, et al. Diagnostic imaging in the evalua-
tion of suspected aortic dissection: old standards and new directions. N Engl J Med
1993; 328(1):35–43.
38. Nienaber CA, Von Kodolitsch Y. Diagnostic imaging of aortic diseases. Radiologe
1997; 37(5):402–409.
39. Moore AG, Eagle KA, Bruckman D, et al. Choice of computed tomography, trans-
esophageal echocardiography, magnetic resonance imaging, and aortography in acute
aortic dissection: international registry of acute aortic dissection (IRAD). Am J Cardiol
2002; 89(10):1235–1238.
40. Nienaber CA, Richartz BM, Rehders T, et al. Aortic intramural haematoma: natural
history and predictive factors for complications. Heart 2004; 90(4):372–374.
41. Yamada T, Tada S, Harada J. Aortic dissection without intimal rupture: diagnosis with
MR imaging and CT. Radiology 1988; 168(2):347–352.
42. Sarasin FP, Louis-Simonet M, Gaspoz JM, et al. Detecting acute thoracic aortic
dissection in the emergency department: time constraints and choice of the optimal
diagnostic test. Ann Emerg Med 1996; 28(3):278–288.
43. 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.
44. 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(3):658–664.
45. Walker PJ, Dake MD, Mitchell RS, et al. The use of endovascular techniques for the
treatment of complications of aortic dissection. J Vasc Surg 1993; 18(6):
1042–1051.
46. Erbel R, Oelert H, Meyer J, et al. Effect of medical and surgical therapy on aortic dis-
section evaluated by transesophageal echocardiography: implications for prognosis
and therapy. Circulation 1993; 87(5):1604–1615.
47. Coady MA, Rizzo JA, Hammond GL, Kopf GS, Elefteriades JA. Penetrating ulcer of
the thoracic aorta: what is it? how do we recognize it? how do we manage it? J Vasc
Surg 1998; 27(6):1006–1016.
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Putting It All Together: Symptoms, Signs, and Images 87
DISCUSSION AND COMMENTARY
Questions for the Authors
How does a frontline physician practically separate out the rare patient with an
aortic dissection from the multitudes of patients with myocardial infarction, or
other causes of chest symptoms?
When patients with acute aortic syndromes present in the classic manner with
typical symptoms and signs, making the correct diagnosis is not difficult. Recall
that the pain of aortic dissection is typically of sudden and dramatic onset,
as opposed to the crescendo or stuttering course seen in myocardial infarction.
Evidence of end-organ malperfusion should alert the clinician to consider acute
aortic dissection. The real challenge for the front-line physician is in suspecting
an acute aortic syndrome in the patient who presents with atypical features. Recall
that women, the elderly, diabetics, and those with prior cardiac or aortic surgery
are more likely to present with atypical features, including nonspecific chest pain
or synocope. An even higher index of suspicion is required when evaluating symp-
toms in these patients, especially when more common diagnoses (e.g., myocardial
infarction) have been ruled out.
How much can fairly be expected of the frontline physicians in recognizing aortic
dissection in a patient presenting solely with abdominal symptoms? Aortic dissec-
tion must be very low down in the differential diagnosis among the myriad of
patients seen in an emergency room with abdominal pain or nausea.
A high index of suspicion for aortic dissection is necessary whenever evaluating
abdominal pain in patients at risk for this type of atypical presentation, especially
when a more obvious diagnosis has not been made. Patients predisposed to pre-
senting with abdominal pain include the elderly patient with hypertension or known
aortic aneurysm or patients who have had prior aortic surgery. The physical exam
should always include careful palpation and auscultation of the abdomen for
pulsatile mass or bruit and a thorough examination of the peripheral vasculature
for pulse deficits should be documented. Laboratory testing may confirm evidence
of end-organ malperfusion, including acute renal insufficiency or mesenteric
ischemia. Appropriate aortic imaging tests should be obtained for any patient with
unexplained abdominal pain that has these risk factors or physical signs.
Do you feel that there is ever a role for going directly to the operating room in a criti-
cally ill patient with suspected aortic dissection even without an imaging study (e.g.,
in a hypotnesive patient with a unilateral pulse deficit)?
When the pretest probability of acute dissection is intermediate or less, the most
easily obtained aortic imaging test should be sought to confirm the diagnosis.
However, acute aortic dissection causing hemodynamic instability is a surgical
emergency that requires rapid surgical intervention. In these patients, withholding
surgery until confirmatory imaging studies are obtained may create an unacceptable
delay in delivering life-saving treatment. When the diagnosis of acute dissection
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88 Raghupathy and Eagle
is highly likely based on presenting characteristics and physical signs, the cardiac
and surgical consultants should be notified immediately, and an operating room
should be emergently prepared to minimize logistical delays. When available, the
ideal imaging study in this situation is a portable transesophageal echocardiogram
(TEE), which can follow the patient into the operating room and rapidly confirm
the diagnosis just prior to surgery.
How does one identify the “reverberation artifact” that can cause a false
appearance of an aortic flap on echocardiography (TTE or TEE)? What are the
distinguishing characteristics?
Reverberation artifact usually mirrors an adjacent structure (i.e., a calcified seg-
ment of aortic wall) and “moves” in parallel to this reference structure. In contrast,
a true aortic dissection flap moves independently of surrounding structures, often
undulating in more than one plane. To better distinguish artifact from an actual
dissection flap, one should examine the structure, utilizing multiple viewing
angles, to ensure the abnormality makes sense anatomically.
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89
5
Epidemiology: Incidence,
Prevalence, and Trends
John A. Elefteriades
Section of Cardiothoracic Surgery, Yale University,
New Haven, Connecticut, U.S.A.
John A. Rizzo
Department of Epidemiology, State University of New York at Stony Brook,
Stony Brook, New York, U.S.A.
INTRODUCTION
For multiple reasons, it is a challenge to assess the epidemiology (incidence and
prevalence) of aortic aneurysms and their corresponding acute aortic diseases.
The methodological challenges include the following:
1. Referral center bias. Tertiary referral centers that specialize in treatment
of aortic diseases receive patients from a broad geographic territory.
The number of patients treated at such centers does not, therefore,
directly reflect the incidence or prevalence of aortic diseases in the
hospital environs.
2. Unknown presence of aneurysm. Unlike many other cardiac and pulmonary
diseases, aneurysms produce no specific symptoms in over 95% of affected
patients. Thus, large pools of patients go undiagnosed unless they have
undergone screening imaging studies—almost always for other purposes.
3. Misdiagnosis as myocardial infarction. Frequently, patients who enter
an emergency room with aortic rupture or dissection and promptly die
are misdiagnosed as “heart attack” (1). This is understandable. The patient
presents with chest pain, he often loses consciousness, instability of vital
signs ensues, and the patient dies in electromechanical dissociation.
Thus, routine autopsies on patients dying in the emergency room are
SECTION III: THE BIOLOGY OF ACUTE AORTIC CONDITIONS
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90 Elefteriades and Rizzo
required to ascertain the true incidence of death from aortic rupture
and dissection. Anagnostopoulos has said, “Acute aortic dissection is
the most common catastrophe involving the aorta” (1). This statement
is based on the recognition that autopsy studies disclose that many
patients thought to have died from myocardial infarction have actually
succumbed from aortic dissection (1,2).
4. Confusion in terminology. It is common for three related but distinct
entities—acute aortic transection, rupture of aortic aneurysm, and aortic
dissection—to be confused, both in substance and in terminology
(Fig. 1). Acute aortic transaction is a traumatic phenomenon, with
circumferential tearing of the wall of the aorta, without propagating
dissection. The aortic wall is intrinsically normal and resistant to the
dissection process. Rupture of aortic aneurysm is self-explanatory;
however, confusion in terminology may arise if an acute aortic transac-
tion or an acute aortic dissection happens to rupture—a common even-
tuality. Acute aortic dissection refers to the very specific process of
separation of layers of the aortic wall discussed fully in other portions
of this book. For dissection to occur, the aortic wall must nearly always
be affected by structural disease of the media. These three conditions—
acute aortic transaction, ruptured aortic aneurysm, and acute aortic
dissection—are frequently confused, leading to difficulties in categori-
zation for epidemiologic purposes.
5. Limitations of administrative databases. Administrative data bases—such
as those maintained by state and Federal governments, by insurers, and
by hospitals and hospital systems—rarely are so detailed as to accommo-
date the subtleties of the factors enumerated here. Thus, data on incidence
and prevalence arising from such databases are inherently flawed.
Despite these considerable methodological limitations, multiple studies have
assessed the epidemiology of abdominal and thoracic aortic aneurysm (3–17).
Relatively self-contained populations in Olmstead County (Minnesota) and Upsalla
Figure 1 Three commonly confused disorders. (A) Acute aortic transaction. (B) Degenerative
aneurysm of the descending aorta. (C) Acute aortic dissection. Confusion in terminology
wreaks havoc with epidemiologic studies. Source: From Ref. 20.
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Epidemiology: Incidence, Prevalence, and Trends 91
(Sweden) (Fig. 2) have contributed immensely to our understanding of the epide-
miology of aneurysm disease. Nonetheless, given the methodological issues
discussed earlier, the incidence and prevalence figures for aortic aneurysms should
best be viewed as rough approximations of the true rates. The most important
findings are summarized by category in the accompanying Table 1.
PREVALENCE
The most recent data available from the Centers for Disease Control indicates
that aneurysm disease (abdominal and thoracic) is the 17th most common cause of
death in all individuals and the 15th most common in individuals aged over 65,
accounting for 14,810 and 12,040 deaths annually in these two groups, respectively
(3) (Table 2). Note that aneurysm causes more deaths than Human Immunodeficieny
Virus (HIV) disease. These figures almost certainly represent underestimates, not
only for the reasons enumerated earlier in this chapter, but also because many
aneurysm-related deaths (especially for the ascending aorta) are likely included in
the group classified as “cardiac” and not tabulated in the aneurysm figures. In fact,
experts have suggested that 30,000 to even 60,000 aneurysm related deaths per
year in the United States per year represent a more reasonable estimate (1).
Abdominal aortic aneurysm (AAA) affects about 5% of individuals over
65 years of age. The prevalence is considerably higher in men than in women (4–6).
INCIDENCE
The incidence of ruptured AAA is about 10 per 100,000 patient years (7). For men
in their 60s, this rate rises to about 50 per 100,000 patient years. For men in their
Figure 2 Note increased incidence of ruptured abdominal aortic aneurysm in total popu-
lation of Malmo, Sweden, between 1971 and 2004. Source: From Ref. 7.
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