(Chest. 2001;120:1340-1346.)
© 2001
American College of Chest Physicians
Aortic Intramural Hematoma*
An Increasingly Recognized and Potentially Fatal Entity
Neilander S. Sawhney, MD;
Anthony N. DeMaria, MD, FCCP and
Daniel G. Blanchard, MD
*
From the Division of Cardiology, Department of Medicine, University of California, San Diego School of Medicine, and UCSD Medical Center, San Diego, CA.
Correspondence to: Daniel G. Blanchard, MD, UCSD Medical Center, 200 W. Arbor St, #8411, San Diego, CA 92103-8411; e-mail: dblanchard{at}ucsd.edu
 |
Abstract
|
|---|
Aortic intramural hematoma (IMH) is related to but is
pathologically distinct from aortic dissection. In this potentially
lethal entity, there is hemorrhage into the aortic media in the absence
of an intimal tear. Although intimal disruption is not present, the
prognosis is similar to that of classic aortic dissection; therefore,
early diagnosis is critical. In this review, symptoms and prognosis of
aortic IMH are discussed, as well as current diagnostic techniques and
therapy.
Key Words: aortic dissection aortic intramural hematoma transesophageal echocardiography
 |
Introduction
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Aortic
dissection is a life-threatening medical condition, and immediate
diagnosis and treatment are crucial. The vast majority of aortic
dissections begin with a primary intimal tear. Blood flow enters the
media through this tear and propagates distally, creating a false
lumen. A less common variant of aortic dissection exists, however,
termed aortic intramural hematoma (IMH). In this entity, hemorrhage
occurs within the aortic wall in the absence of initial intimal
disruption. Although its existence has been validated by surgical and
pathologic examination, the lack of an intimal flap has led to
underdiagnosis by aortography in the past.1
2
3
4
5
6
With recent
advances in imaging techniques, IMH is now increasingly recognized. The
limited data available suggest that the clinical course of IMH mimics
that of acute aortic dissection, and mortality rates are similar. Thus,
a familiarity with IMH is useful during the evaluation of acute chest
pain. Herein we will review the pathogenesis, natural history,
diagnosis, and treatment of IMH.
 |
Pathogenesis
|
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The initial event in aortic dissection has long been a point of
controversy. It is unclear who first described aortic dissection, but
references are found as far back as 1761 in Nicholls description of
the autopsy of King George II of England.7
The term
aortic dissection is often credited to Laennec in
1826.8
9
Laennec and the scholars of his time believed
that the intimal tear was the inciting event that allowed blood to
enter the media and cause distal dissection. Indeed, autopsy
examinations have revealed that most dissections have intimal tears and
that these tears usually occur where shear forces are
greatest.10
11
An alternate theory of pathogenesis evolved in the early 1900s when
several authors independently described the existence of IMH (that is,
a hemorrhagic dissection of the media without an intimal tear). Of
these authors, Krukenberg12
first proposed that a rupture
of the vasa vasorum initiated the process of aortic dissection.
Gore8
championed this view in the 1950s and suggested that
underlying medial degeneration predisposed the vasa vasorum toward
hemorrhage and that IMH "... may be the usual rather than the
uncommon mechanism of hemorrhagic dissection." In support of this
theory, intimal tears are not always present in cases of aortic
dissection. There are also well-documented cases of acute IMH rupturing
through the intima and evolving into more typical dissections during
the course of treatment.1
5
13
14
15
16
17
18
19
20
21
22
23
 |
Natural History
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We have a limited knowledge of the natural history of IMH, as much
of the data comes from case reports and small retrospective series. In
a review of 505 autopsy cases of aortic dissection from 1933 to 1954,
Hirst et al9
found a 4% incidence of IMH. In 1982, an
autopsy study10
noted that 13% of patients with a
diagnosis of aortic dissection had IMH. Similarly, clinical
series1
13
14
15
16
17
19
have shown that 13 to 27% of patients
with a diagnosis of aortic dissection in fact had IMH.
IMH is difficult to distinguish from classic dissection on purely
clinical grounds. The most common initial presentations of IMH are
chest pain (50 to 74%) and intrascapular back pain (44 to
84%).13
14
15
16
17
18
19
20
21
Other more rare presentations include
neurologic or vascular complications (such as syncope or transient
ischemic attack), hoarseness, paraplegia, mesenteric ischemia, and
acute renal failure. Patients with IMH are typically elderly (mean age,
66 years) with a history of hypertension.13
14
15
16
17
18
19
20
21
Unlike
classic aortic dissection, the ratio of men to women appears
equal.13
14
15
16
17
18
19
20
21
Other traditional risk factors for aortic
dissection, such as bicuspid aortic valve, Marfan syndrome, and
collagen vascular disease, have been distinctly uncommon in case series
of IMH. Several series13
14
15
16
17
18
19
20
21
have noted the development of
IMH following trauma. Proximal aortic involvement occurs in 40% of
cases (range, 20 to 60%).
IMH has a variable clinical course. While some patients have limited
hemorrhage and respond well to medical therapy, IMH may progress to
classic dissection (ie, with intimal disruption) in up to
33% of cases.1
13
14
17
20
21
22
23
24
25
26
27
28
29
30
31
32
33
IMH of the ascending aorta
maysimilar to classic dissectionrupture through the adventitia and
cause pericardial effusion, hemothorax, and mediastinal hemorrhage.
These vascular catastrophes largely account for the observed acute
mortality from IMH (up to 30%).13
14
15
16
17
18
19
20
21
The mortality from
proximal aortic IMH is higher than that of distal IMH (34% vs 14%),
and deaths tend to occur within the first 24 to 72 h after
hospital admission (Table 1
).13
14
Some medically treated patients with IMH may have complete
resolution.1
2
5
16
17
18
22
24
25
32
Yamada et
al2
followed up 10 survivors of IMH and found that 8
survivors had normal chest CT scan findings within 1 year. Ide et
al22
performed serial CT scans in 27 patients and
demonstrated a dynamic nature of IMH. At 2 months, 19 of 27 patients
with IMH showed resolution, but in 2 other patients, late progression
to aortic aneurysm occurred. Furthermore, Ide et al22
described three patients in whom IMH converted to classic aortic
dissection within 4 to 40 months of presentation.
Sueyoshi et al33
followed up 32 patients with IMH with
serial CT imaging at weekly intervals in the first month followed by
further imaging two to three times per year. Their results were similar
to those of Ide et al,22
with 11 of 32 patients showing
resolution by 24 months and 18 of 32 patients showing aneurysms
eventually evolving in the affected portions of the
aorta.22
23
Interestingly, the authors33
noted the development of "ulcer-like projections" on the aortic
intima during the follow-up period. Of 21 patients with these
projections, saccular aneurysms developed in 12 patients at a mean of
45 days following the initial event, with an average growth in aortic
diameter of 1.2 cm/yr. These authors hypothesized that IMH causes
structural weakness of the aortic walleven after
resolutionaccounting for the high incidence of late
aneurysms.33
 |
Diagnosis
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Problems in defining the natural history of IMH stem from
difficulty in diagnosing the entity. While the diagnosis of aortic
dissection can be challenging, detection of IMH has been even more
problematic. For many years, aortography was the only method available
to diagnose aortic dissection premortem, and many still consider
aortography the "gold standard." Studies have shown, however, that
aortography is not as accurate in the diagnosis of dissection as once
believed. In a prospective study of 126 patients with aortic
dissection, Erbel et al4
showed aortography to have a
sensitivity of 88% and a specificity of 94%. Similarly,
others28
29
have reported the sensitivity of aortography
to be 77 to 87%. Aortography relies primarily on direct visualization
of an intimal flap or double lumen for diagnosis, although indirect
signs such as aortic thickening and compression of the true lumen by
the false lumen also may be present. By definition, IMH has neither an
intimal flap nor intraluminal flow.28
29
30
Newer imaging techniques have markedly enhanced our ability to detect
IMH. These modalities, including MRI, spiral CT, and transesophageal
echocardiography (TEE), have basically replaced aortography in the
evaluation of IMH. Unquestionably, MRI yields strikingly accurate
images of the aorta. It is noninvasive and requires no intravascular
contrast. Several studies30
31
place the sensitivity of
MRI for detection of aortic dissection at 98 to 100% and the
specificity at 100%. MRI also detects complications of aortic
dissection and IMH, such as pericardial effusion, hemothorax, and
hemomediastinum. Although experience with IMH is considerably less than
with classic dissection, MRI has accurately diagnosed IMH in several
series.1
3
5
13
16
25
Clinically, the technique has
several drawbacks, including difficulty with critically ill patients
and those with metallic prosthetic valves or permanent pacemakers.
Finally, most MRI scanners cannot accurately quantify coronary
involvement or aortic regurgitation.
With MRI, IMH is characteristically seen as a focal thickening of the
aortic wall in the absence of dissection (Fig 1
). There is usually minimal compression of the aortic lumen, and of
course no intimal flap. An acute aortic IMH has an isodense intensity
on T1-weighted images and has a high signal intensity on T2-weighted
images. The signal intensity evolves over time, as oxyhemoglobin is
converted to methemoglobin, resulting in an increased signal intensity
on T1-weighted images in subacute IMH. Dynamic phase-contrast imaging
can support no flow within the aortic wall.2
5
13
17
25

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Figure 1. T2-weighted transverse MRI of descending aortic
IMH. The descending aorta is dilated, and the wall is thickened. The
signal from the wall is abnormally intense, consistent with IMH.
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|
CT has successfully identified IMH in several
reports.1
2
3
5
13
16
18
22
23
Compared to MRI, CT appears
somewhat less accurate in identifying intimal flaps, which may lead to
the overdiagnosis of IMH in cases of dissection.30
34
35
As the treatments for dissection and IMH are currently similar, however
(see below), this point may be clinically moot. Like MRI, CT cannot
identify coronary involvement or aortic regurgitation, and it has a low
sensitivity for detection of branch artery occlusions. Management of an
intubated or critically ill patient is often easier with CT than with
MRI, but as with angiography, CT carries with it the inherent risks of
intravascular contrast.27
A CT image (with intravascular
contrast) is shown in Figure 2
. The intramural hematoma is seen as an area of low attenuation within
the aortic wall on contrast-enhanced CT (and of high attenuation on
noncontrast CT). Absence of contrast within the aortic wall helps
differentiate IMH from a classic aortic dissection (where contrast
enters the false lumen). Distinguishing IMH from aortic dissection with
thrombosis of the false lumen remains problematic. In addition, there
may be difficulty in differentiating IMH from penetrating aortic ulcer
with adherent intraluminal thrombus. In this regard, the presence of
intimal calcium often can be used to distinguish intramural
(subintimal) hematoma from intraluminal
thrombus.2
26
32
33

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Figure 2. Contrast-enhanced CT image of aortic IMH. The
ascending aorta is dilated, and the walls of the ascending and
descending aorta are abnormally thickened (due to IMH). Contrast is not
seen within the aortic media, and an intimal flap is absent.
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|
With the advent of TEE, echocardiography has become an important
imaging modality for the diagnosis of aortic dissection and IMH. Early
studies of transthoracic echocardiography were limited by poor image
quality, especially in cases of distal aortic dissection. TEE has
allowed better visualization of the aorta, and has a sensitivity and
specificity of about 95% for detection of dissection (with multiplane
probes).4
14
29
34
36
37
Unlike CT, TEE is remarkably
accurate in the detection of intimal flaps, and therefore is an
accurate technique for differentiating dissection from
IMH.14
16
17
TEE is accurate in the detection of aortic
regurgitation, pericardial effusion/tamponade, and proximal coronary
artery involvement.14
27
36
In addition, TEE yields a
diagnosis more quickly than MRI28
and is often easier to
perform in critically ill and/or intubated patients than either CT or
MRI.
The entities that must be distinguished from IMH are aortic aneurysm
with associated thrombus, dissection with thrombosed false lumen, and
severe aortic atherosclerosis (with or without penetrating aortic
ulceration).1
14
15
16
The typical TEE findings of IMH
include a focal or diffuse thickening of the aortic wall in the absence
of an intimal flap or any communication between the aortic lumen and
IMH (Fig 3
, top and bottom). This finding distinguishes IMH
from a dissection with thrombosed false channel. The luminal wall of
the aorta is typically curvilinear and smooth in patients with IMH, as
opposed to the rough, irregular borders seen with aortic
atherosclerosis and penetrating ulcer (Fig 4 ). In addition, IMH tends to be homogeneous in appearance, while
atheroma is often heterogeneous, speckled, and calcified on ultrasound
imaging. Differentiation between IMH and aortic aneurysm with
associated thrombus can be challenging, but the presence of a smooth
intimal border next to the lumen (as opposed to thrombus with an
irregular border interposed between the lumen and intima) favors the
diagnosis of IMH or a thrombosed false lumen (Fig 3
, 4)
.38

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Figure 3. Top: TEE image of IMH (arrows)
limited to the lateral wall of the aorta (AO). An intimal flap is
absent. The IMH is relatively homogeneous in echocardiographic density
and is void of calcification. Bottom: TEE image of
extensive aortic IMH. As in Figure 3
, top, intimal
calcification is absent and the echocardiographic density of the
hematoma is relatively homogeneous.
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Figure 4. TEE image of a large atherosclerotic plaque in the
descending thoracic aorta. The plaque (arrow) protrudes into the aortic
lumen, has an irregular border, and is heterogeneous in
echocardiographic density.
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A relatively new diagnostic tool that has been described for the
diagnosis of IMH is intravascular ultrasound (IVUS) as an adjunct to
aortography.3
19
39
The largest study19
involving IVUS included eight patients with a diagnosis of IMH,
including four patients with diagnoses missed by other imaging
procedures such as angiography and TEE. Unfortunately, IVUS also
carries many of the risks of angiography, and its role in the diagnosis
of IMH is not yet well defined.
 |
Treatment
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Lacking data from clinical trials, treatment of IMH has been
mostly empiric. Most authorities1
3
6
13
14
40
currently
recommend treatment of IMH similar to that of classic aortic
dissection, with early surgery for patients with proximal IMH and
medical management for patients with distal IMH.
Others16
17
22
favor a conservative approach, with initial
medical management for all patients in stable condition.
To our knowledge, the only study to address therapy prospectively was
published by Kang et al16
; these investigators suggested
that IMH might have a more favorable prognosis than aortic dissection,
as the hematoma is "noncommunicating" with the aortic lumen. In
this study,16
27 patients with a diagnosis of IMH were
initially treated with medical management. Seven patients had proximal
IMH; of these seven patients, three patients eventually required
surgery. Twenty patients had distal IMH, and only 1 patient required
surgery. There was no mortality in the proximal dissection group; the
only fatality occurred in a patient with distal IMH treated medically.
The results and patient outcomes in this study16
are in
stark contrast to the majority of published series on IMH (Table 1)
,
which have shown much higher mortality rates overall for IMH. On
review, however, the results of these studies are quite variable.
Treatment in these reports was not randomized, and so a selection bias
is likely (for example, the sickest and most moribund patients may have
been treated medically rather than surgically). Although Kang et
al16
attempted to avoid a selection bias, the majority of
patients followed up in their study presented > 48 h after symptom
onset. Given the high early mortality of aortic dissection and IMH, the
population in the study of Kang et al16
may have excluded
the sickest patients (leaving a group with a somewhat better
prognosis). Additional possible explanations for the disparity between
published series include differences in patient populations and
comorbidities, rapidity of diagnosis, and accuracy of the diagnostic
technique(s) employed. Despite the differences between reports, the
cumulative data are instructive. Total cumulative mortality from IMH
(in the 11 studies listed in Table 1 ) was 21%. Mortality in proximal
IMH cases was 34% (47% for medical management and 24% for surgically
management). Mortality in distal IMH cases was 14% (13% with medical
management, 15% with surgery).
Of interest, Ide et al22
noted that those
patients with IMH who progressed to classic dissection tended to have
aortic diameters > 5 cm. Ide et al22
suggested that this
may be a way to identify patients at risk for dissection and chronic
aneurysm formation. Kaji et al41
reported similar
findings. Thus, the optimal treatment for IMH is not completely clear.
In some patients, medical treatment of IMH with antihypertensives and
negative inotropes appears adequate, but in other patients a surgical
approach may be preferable. Surgical options have usually involved the
placement of Dacron grafts, although resection of the affected aorta
with end-to-end anastomosis (without graft insertion) has been
described.42
While endovascular stenting is emerging as a
treatment option for type-B dissections,43
it has not been
described in the treatment of IMH. Given the lack of an intimal flap,
it is unclear what benefit endovascular stenting would have in the
treatment of IMH.
In the absence of definitive clinical trials, we believe it is prudent
to treat ascending aortic IMH surgically and descending aortic IMH
medically (assuming there is no evidence of rupture or compromise of
organ perfusion). The fact that IMH has a high incidence of late
complications and aneurysm formation underscores the need for periodic
aortic imaging after successful medical therapy.
 |
Conclusion
|
|---|
Aortic IMH is a difficult diagnosis that requires a high index of
suspicion. TEE is an excellent imaging modality for diagnosis,
especially in critically ill patients.1
4
14
15
16
25
27
MRI
is also very accurate and is useful in patients in stable
condition.31
As scanning times decrease, the role of MRI
will likely increase. CT can diagnose aortic dissection reasonably
well, but it may not be completely reliable in distinguishing IMH from
classic aortic dissection (as noted, the clinical importance of this
point is currently unclear). CT also gives less information regarding
complications and prognosis than TEE or MRI.4
27
35
37
As
no single imaging modality is optimal for IMH, there may be utility in
combining imaging modalities to confirm the diagnosis.
It is clear that IMH has a high rate of mortality and morbidity.
Although a recent report40
suggested an improved prognosis
of IMH over aortic dissection, survivors of IMH are at significant risk
for progressive aortic abnormalities, including aortic rupture,
aneurysm, and ulceration.1
2
22
26
33
The optimal therapy
for IMH is uncertain and will remain so until randomized trials are
performed. Until then, we prefer to perform immediate surgery in
patients with proximal IMH (as with classic acute ascending aortic
dissection). In patients with descending IMH (without rupture or
compromised end-organ perfusion), we believe that medical therapy is
appropriate. In this latter group, serial imaging of the aorta is
recommended, as aneurysm formation is not uncommon.
After submission of this review, a retrospective analysis of very
elderly patients with ascending aortic IMH was
published.44
Compared to patients with type-A aortic
dissections, the group with ascending IMH had a somewhat better
prognosis. The authors suggested that supportive medical treatment with
frequent follow-up imaging may be a reasonable alternative to surgery
in the very old patient group with ascending aortic IMH.
 |
Footnotes
|
|---|
Abbreviations: IMH = intramural hematoma;
IVUS = intravascular ultrasound; TEE = transesophageal
echocardiography
Received for publication September 27, 2000.
Accepted for publication January 24, 2001.
 |
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