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291.e4 PART IV Noncoronary Diseases: Diagnosis and Management
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28
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Acute Aortic Syndromes:
Diagnosis and Management
Peter C. Spittell
OUTLINE
Introduction, 292
Acute Aortic Dissection, 292
Pathogenesis, 292
Predisposing Factors, 293
Classification, 293
Clinical Features, 293
Diagnosis, 294
Management, 297
Penetrating Aortic Ulcer, 298
Pathogenesis, 298
Predisposing Factors, 298
Clinical Features, 298
Laboratory Findings, 298
Diagnosis, 299
Management, 299
Aortic Intramural Hematoma, 300
Pathogenesis, 300
Classification, 300
Clinical Features, 300
Diagnosis, 300
Management, 300
INTRODUCTION
Acute thoracic aortic syndromes comprise a spectrum of medical
and surgical emergencies, including acute aortic dissection,
penetrating aortic ulcer, and aortic intramural hematoma. All
of these conditions are potentially life threatening and warrant
prompt diagnosis and emergent management. The clinical
presentation of acute aortic syndromes is highly variable, ranging
from occult disease to classic clinical presentations. Numerous
etiologic factors, acting singly and in combination, have been
identified. Diagnosis is possible by noninvasive imaging in the
majority of patients, but in some patients complementary
noninvasive tests (transesophageal echocardiography [TEE],
computed tomography [CT], and magnetic resonance imaging
[MRI]) are required for diagnosis. The management of acute
aortic syndromes continues to be a therapeutic challenge, while
diverse surgical and percutaneous strategies for the treatment
of aortic syndromes are continuously evolving. As a result of
increasing knowledge and better management strategies in this
area, the outcomes of patients treated for acute aortic syndromes
have improved. Therefore, awareness of the clinical features of
acute aortic syndromes and familiarity with currently available
diagnostic techniques is basic to their effective treatment.
ACUTE AORTIC DISSECTION
Acute dissection of the thoracic aorta is one of the most common
catastrophic aortic conditions encountered in clinical practice.
The incidence of aortic dissection has been reported to be
approximately 2.9 per 100,000 per year.
presentations of aortic dissection, in combination with a mortality
rate in untreated cases as high as 1% per hour during the first
48 hours after the onset of symptoms, underscore the importance
of a high index of suspicion and prompt diagnosis and therapy.
Noninvasive testing (TEE, CT, and MRI) allows an accurate
diagnosis to be made in the majority of patients.
treatment exists so that future improvements in initial and
long-term survival in acute aortic dissection depend on increased
clinical awareness, rapid noninvasive diagnosis, and the early
institution of appropriate medical and/or surgical therapy.
1
The variable clinical
5,6
Effective
2–4
Pathogenesis
Aortic dissection originates at the site of an intimal tear in more
than 95% of patients.2 The resultant intimal tear exposes the
media to pulsatile aortic flow, creating a second or “false” aortic
lumen that then dissects in the outer layer of aortic media,
propagating distally and, occasionally, proximally.
Ascending aortic dissections are almost twice as common as
descending dissections. Some 50% to 65% of aortic intimal tears
originate in the ascending aorta and approximately 20% to 30%
of intimal tears originate in the vicinity of the left subclavian
7
ar tery.
Once initiated, the dissection usually extends distally and,
occasionally, proximally for a variable distance. As the dissecting
process encounters branches of the aorta, it may pass around
their origins, extend into their walls, or occlude them.2 Reentry
of the dissection through a second, more distal intimal tear may
occur, usually in one of the iliac arteries. External rupture of
292

CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 292.e1
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Keywords
aorta
aortic dissection
penetrating aortic ulcer
aortic intramural hematoma
echocardiography

CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 293
Type A (proximal) Type B (distal)
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the dissecting process into the pericardial space is the most
common cause of death in aortic dissection. Acute congestive
heart failure, usually due to aortic regurgitation, is the second
most common cause of death.
Predisposing Factors
Multiple risk factors for aortic dissection have been identified,
the most common being advanced age, systemic hypertension,
congenital abnormalities of the aortic valve, and heritable disorders of connective tissue.
Aortic dissection most frequently affects patients in the fifth
to seventh decades of life (mean age, 63 years) and is more
common in men (65.3% male).7 In patients younger than 40
years, the incidence between men and women is equal due to
the occurrence of aortic dissection in women during pregnancy,
with approximately 50% of all aortic dissections occurring during
the third trimester of pregnancy.
Hypertension is present in 70% of type B dissections, but
only in 25% to 35% of type A dissections.
8
7,9,10
Hypertension may
play a role in initiating the intramural hematoma along with
having a direct weakening effect on the aortic media.10 The
causative role of systemic hypertension is further supported by
the finding that coarctation of the aorta predisposes to aortic
dissection.
11
Other major risk factors for aortic dissection, especially
proximal aortic dissection, are congenitally bicuspid or unicommissural aortic valves
7,9,10
and the genetically mediated collagen
disorders such as Marfan syndrome, Loeys Dietz syndrome, and
Ehlers-Danlos syndrome.
7,12
Additional predisposing factors
for acute aortic dissection include preexisting aortic aneu-
7,13
rysm
and a positive family history (in as many as 19% of
patients).
Iatrogenic aortic dissection is an uncommon but potentially
serious complication of invasive angiographic procedures and
cardiac surgery.
10,14–17
Catheter-induced dissection can originate
at any location; the majority are retrograde dissections and tend
to decrease in size over time owing to thrombosis of the false
lumen, whereas anterograde dissections tend to persist on followup. Nearly all iatrogenic aortic dissections can be treated medically
with serial clinical examinations and noninvasive testing used
to identify those in need of surgical therapy.
15
Cardiac surgical procedures complicated by aortic dissection
include those that require cross-clamp or cannulation of the
ascending aorta, such as aortic valve replacement and/or coronary
artery bypass grafting.
10,18,19
Dissection can arise at the site of
ascending aortic cannulation, aortosaphenous vein anastomosis,
aortic cross-clamp, or as a result of direct arterial injury. Dissection
in association with these procedures usually occurs intraoperatively and is promptly diagnosed and treated, but chronic dissection in the postoperative period has been reported.
20
Aortic dissection has also been reported in association with
inflammatory diseases (giant cell aortitis,
10,21
Takayasu aortitis,
rheumatoid arthritis, syphilitic aortitis, systemic lupus erythematosus,22 Noonan syndrome,23 Turner syndrome,24 fibromuscular
dysplasia, annuloaortic ectasia, aortic coarctation, cocaine use,
25,26
methamphetamine use, polycystic kidney disease,27 polyarteritis
nodosa,28 trauma,29 and high-intensity weight lifting.
30
Fig. 28.1 Classification of aortic dissection. Type I refers to a
primary tear in the ascending aorta and dissection involving the
aortic arch and descending thoracic aorta for a variable distance.
Type II refers to dissection involving only the ascending aorta.
Type III refers to a primary tear distal to the subclavian artery
origin, extending distally for a variable distance. (Modified from
DeBakey ME, Henly WS, Cooley DA, et al. Surgical management
of dissecting aneurysms of the aorta. J Thorac Cardiovasc Surg.
1965;49:130–149.)
Classification
There are two main anatomic systems—the DeBakey and Daily
(Stanford) systems—used to classify aortic dissection. The
DeBakey system31 is based on the site of origin of the dissection
and recognizes three types of dissection (Fig. 28.1). For clinical
purposes, because types I and II have a similar prognosis, the
more widely used Stanford system classifies dissections that
involve the ascending aorta as type A, and all other dissections
as type B.
Dissections are categorized as acute if the diagnosis is made
within 2 weeks of symptom onset and as chronic if more than
2 weeks have elapsed. The distinction is important owing to the
fact that approximately 65% to 75% of patients with untreated
aortic dissection die in the first 2 weeks after the onset of
symptoms.
Intimal tear without hematoma is an uncommon variant of
aortic dissection characterized by a localized intimal tear exposing
the underlying media or adventitial layers to pulsatile aortic
flow. There is no progression or separation of the medial layers.
Clinical Features
Owing to variable involvement of the aorta and its branches by
the dissecting process, the patient with acute aortic dissection
may have clinical manifestations of ischemia of various organ
systems, singly or in combination, and symptoms and signs of
cardiac disease. The diverse presentations of aortic dissection
can make diagnosis difficult, and misdiagnosis commonly
occurs.
of aortic dissection and in medical and surgical therapy, up to
Type I
32,33
2
2,10
Despite major advances in the noninvasive diagnosis
Type II Type III
34

294 PART IV Noncoronary Diseases: Diagnosis and Management
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55% of patients in reported series die without a correct antemortem diagnosis.
1,2,10,35,36
For these reasons, a high index of
clinical suspicion for acute aortic dissection in any likely setting
is imperative.
The classic presentation of a patient with acute aortic dissection, occurring in more than 70% of patients, is the sudden
onset of severe pain, usually beginning in the anterior chest,
radiating to the back, and moving distally as the dissection
progresses.
1,32,37
Chest pain is significantly more common in
patients with type A dissection (79% in type A vs. 63% in type
B dissection). In contrast, both back pain and abdominal pain
are significantly more common with type B aortic dissection
over type A: 64% versus 47% for back pain and 43% versus 22%
for abdominal pain.7 Although painless dissection occurs in 14%
to 21% of patients, it is relatively uncommon.
2,10,38
Patients with
painless aortic dissection tend to be slightly older, have a prior
history of diabetes, aortic aneurysm, or prior cardiac surgery,
and more often have type A dissection.
Patients with acute aortic dissection often appear to be in
shock, although hypertension is present in one-half to two-thirds
of cases, especially with type B dissection.7 Hypotension is more
common in type A dissection and may result from severe aortic
regurgitation and/or rupture of the dissection into the pericardial
space with resultant cardiac tamponade or, less commonly, the
pleural space or mediastinum.
A cardiac murmur may be present, usually at the cardiac base,
and may be systolic, diastolic, or both. A diastolic decrescendo
murmur of aortic regurgitation indicates involvement of the
ascending aorta and is heard in one-half to two-thirds of patients
with type A aortic dissection.7 Congestive heart failure, when
present in association with proximal aortic dissection, is most
often due to severe aortic regurgitation,
9,32
but cases of congestive
heart failure due to rupture of the dissecting process into the
right or left atrium or right ventricle have also been reported.
39–41
Myocardial infarction, most commonly inferior infarction, occurs
in 5% of patients and is due to compromise of either coronary
ostium by a hematoma or intimal flap. Peripheral pulse deficits
are noted in 19% to 30% of patients,
3,7,32
more commonly with
type A aortic dissection, and are associated with a higher rate
of in-hospital complications and mortality.42 Pulse deficits may
be transitory owing to oscillation of the intimal flap or distal
reentry of the hematoma into the true lumen. Acute lower
extremity ischemia, with or without chest pain, as a result of
dissection extending into the iliac arteries occurs in 6% to 12%
3,43,44
of patients
and may provide an important clue to the
diagnosis.43 Other cardiovascular findings include a difference
in systolic blood pressure between the arms (>20 mm Hg),
tachycardia, pericardial friction rub, arterial bruits, pulsus paradoxus, and cardiac tamponade.
Syncope occurs in 5% to 10% of patients with aortic dissection
and is an important event, as it is associated with a worse
prognosis. Syncope most commonly results from either rupture
of the dissecting process into the pericardial space, producing
cardiac tamponade or involvement of the brachiocephalic arter-
7,10,32
ies.
Less commonly, rupture occurs into the left pleural space,
producing a left hemothorax.10 Neurologic deficits—including
cerebrovascular accident, disturbances of consciousness, ischemic
paraparesis, and ischemic peripheral neuropathy—may also
1,7
occur.
Other less frequent findings that occur in association with
acute aortic dissection include Horner syndrome, a pulsatile
sternoclavicular joint,45 vocal cord paralysis, hemoptysis,46 superior
vena cava syndrome,47 upper airway obstruction,48 hematemesis,49
pleural effusion, unilateral pulmonary edema,50 signs of mesenteric
or renal infarction, fever,51 and deep venous thrombosis.
As a general rule, aortic dissection should always be considered
in the differential diagnosis of a patient with unexplained syncope,
stroke, congestive heart failure, acute arterial occlusion, or an
abnormal aortic contour on chest radiography, even in the absence
of chest pain.
Diagnosis
Although aortic dissection may be suspected from the initial
history and physical examination, the correct clinical diagnosis
is made in less than 50% of patients. The clinical diagnosis of
aortic dissection can be improved upon by utilizing the aortic
dissection detection risk score, a clinical tool incorporating
high-risk conditions, high-risk pain features, and high-risk
examination features that can be used to estimate the pretest
probability of disease and rapidly identify high-risk patients,
facilitating prompt evaluation and treatment. The aortic dissection
detection risk score has a sensitivity of 95.7%.
Although routine blood tests are nonspecific in acute aortic
dissection, a D-dimer less than 500 ng/mL is highly predictive
for excluding aortic dissection in low-risk patients.53 An aortic
dissection detection risk score less than or equal to 1 and D-dimer
less than 500 ng/mL accurately ruled out acute aortic dissection
with a low failure rate (sensitivity 98.7%).
The most common electrocardiographic abnormality in
patients with aortic dissection is left ventricular hypertrophy
from chronic systemic hypertension.
3,7
Acute electrocardiographic
changes occur in up to 55% of patients and include ST segment
depression, T-wave changes, and ST segment elevation, in decreasing order of frequency.55 Acute ischemic changes can occur when
one or both coronary ostia become obstructed, either by the
intimal flap or from external compression by the dissecting
hematoma. The electrocardiographic changes of acute pericarditis
may be seen if there has been leakage of blood into the pericardial
space. Heart block resulting from proximal extension of the
hematoma into the area of the atrioventricular node has also
been reported.
56
The chief value of the electrocardiogram is in
distinguishing aortic dissection from acute myocardial infarction,
although the two conditions can coexist.
Chest radiography may be helpful in suggesting the diagnosis
of aortic dissection, with abnormalities of the aortic silhouette
being the most common finding.
7,57
Additional findings include
pleural effusion, mediastinal widening, displacement of intimal
calcification greater than 6 mm inside the outer edge of the
aortic shadow, and the radiographic findings of congestive heart
failure (Fig. 28.2). Nonetheless, it is important to remember that
normal chest radiographic findings (present in 11% to 16%
of patients) do not exclude the diagnosis of aortic dissection.
Confirmation of the diagnosis of aortic dissection requires
cardiovascular imaging that demonstrates the dissection flap
52
54
10
7

CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 295
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A
Fig. 28.2 Chest radiographs in a patient with aortic dissection. (A) Baseline anteroposterior chest
radiograph before presentation with aortic dissection. (B) Chest radiograph 1 year later when the
patient presented with acute aortic dissection. Note the increased diameter of the ascending
aorta and aortic arch and new widening of the superior mediastinum.
B
separating a false lumen from the true lumen. Currently available
noninvasive imaging modalities that are accurate in the diagnosis
of acute aortic dissection include multiplane TEE, CT angiography,
and MR angiography.
Transthoracic echocardiography (TTE) can be very useful in
some patients with suspected aortic dissection (Video 28.1).
When the findings of a dilated aortic root (end-diastolic diameter
>42 mm), widening of the aortic walls (16 to 20 mm for the
anterior wall and 10 to 13 mm for the posterior wall), and a
linear undulating echo representing the intimal flap are present,
the positive predictive value for TTE is 100%.
58–60
Advantages
of TTE are its portability; real-time diagnosis; ability to identify
associated aortic regurgitation; and to assess left ventricular
function, regional wall motion abnormalities, pericardial effusion,
and cardiac tamponade. The diagnosis of cardiac tamponade in
a patient suspected of having aortic dissection deserves special
mention. Echocardiographically guided pericardiocentesis
should be avoided in this setting, as the rapid withdrawal of
pericardial fluid can result in a prompt improvement in left
ventricular systolic function, left ventricular dP/dT, and systolic
blood pressure, producing aortic rupture.
61,62
The patient with
aortic dissection complicated by cardiac tamponade should be
taken emergently to surgery for institution of cardiopulmonary
bypass followed by evacuation of blood from the pericardial
space. Disadvantages of TTE in the diagnosis of aortic dissection
include difficulty in adequately visualizing the descending thoracic
5
and suboptimal echocardiographic windows in patients
aorta
with obesity or chronic obstructive pulmonary disease. Overall,
the sensitivity and specificity of TTE are inferior to TEE, CT,
and MRI in the diagnosis of aortic dissection. Intravenous contrast
agents improve the diagnostic accuracy of TTE in patients with
aortic dissection, achieving a sensitivity and specificity similar
to TEE in type A dissection.63 It should be remembered that a
negative TTE does not exclude aortic dissection.
Multiplane TEE has a sensitivity of 99% and a specificity of
98% in the diagnosis of aortic dissection.64 TEE is portable,
minimally invasive, and can accurately determine the type and
extent of dissection safely in an emergent setting, allowing rapid
triage of patients to either surgical or medical therapy
65,66
(Video
28.2). Color flow Doppler imaging significantly improves the
sensitivity of TEE by allowing visualization of the intimal flap,
dissection entry site, the true and false lumens, presence of
thrombus, mechanism and degree of aortic regurgitation, and
the proximal coronary arteries66 (see Fig. 28.2). In addition,
multiplane TEE provides a comprehensive assessment of left
ventricular systolic function, regional wall motion, pericardial
effusion, and cardiac tamponade. The overall sensitivity of TEE
is comparable with CT,67 MR,
5,68,69
and aortography70 in the
diagnosis of aortic dissection. Current multiplane TEE probes
have largely overcome impediments in the ascending aorta,
although artifacts in this region continue to be a diagnostic
challenge.
CT with intravenous iodinated contrast enhancement is an
accurate noninvasive screening test in patients with suspected
aortic dissection.71 Advantages of CT include ready availability
at most hospitals and improved accuracy with spiral (helical)
CT and electron beam (ultrafast) or multidetector (multislice)
CT.72 CT can reliably demonstrate the intimal flap, pericardial
and pleural effusion, associated mediastinal hemorrhage, and
involvement of the aortic arch vessels and branches of the
abdominal aorta, as well as coronary artery disease5 (Fig. 28.3).
Disadvantages of CT include the need for iodinated contrast
exposure and nonportability, limiting its use in patients with
significant renal insufficiency and in hemodynamically unstable
patients, respectively. In addition, the site of entry is rarely
identified.
Although less commonly used,
7
MRI is a highly accurate
noninvasive technique in the evaluation of patients with suspected
aortic dissection.73 MRI is superior to TEE and CT in detecting
arch vessel involvement and in identifying the anastomosis in
patients managed with surgical therapy and may facilitate
comparison of serial studies.
5,68,74
Gated spin-echo MRI accurately
demonstrates the entry site and intimal flap75 and may be the
optimal method for demonstrating thrombus formation and
entry site location within all segments of the aorta76 (Fig. 28.4).
The ability to obtain oblique and longitudinal planes of a section
makes MRI especially valuable in demonstrating dissection
without intimal tear.
5,69,77
Disadvantages of MRI include cost,

296 PART IV Noncoronary Diseases: Diagnosis and Management
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Fig. 28.3 Computed tomographic scan in a patient with type A
aortic dissection. Note the complex intimal flap seen within the
distal ascending aorta and descending thoracic aorta (arrows),
and differential opacification of the true and false lumens.
Fig. 28.4 Magnetic resonance imaging in the sagittal plane shows
type A aortic dissection with an intimal flap extending into the
distal abdominal aorta.
examination time, reduced availability, nonportability, and
standard contraindications to MRI.
Of the definitive noninvasive imaging modalities in suspected
acute aortic dissection (TEE, CT, MRI), a systematic review of
the diagnostic accuracy of these imaging techniques has demonstrated a pooled sensitivity (98% to 100%) and specificity
(95% to 98%) that is comparable between the three imaging
techniques. Therefore the choice of test depends upon which
imaging modality is readily available at a particular institution
and the hemodynamic stability of the patient.
Fig. 28.5 Aortogram shows a spiraling intimal flap (arrows) in
the ascending aorta and aneurysmal dilation of the ascending
aorta.
Aortography, the traditional definitive diagnostic method in
aortic dissection, is able to localize the site of origin of the dissection and delineate the extent of the dissection and circulation
to vital organs. Diagnostic aortographic features include opacification of the false lumen, deformity of the true lumen by the false
lumen, dilation of the aorta, narrowing or occlusion of branches
of the aorta, and the presence of an intimal flap78 (Fig. 28.5).
Disadvantages of aortography include nonportability, invasive
technique, exposure to ionizing radiation, the use of intravenous
iodinated contrast agents, and an inherent delay in diagnosis.
False-negative aortogram results can occur if there is simultaneous
and equal opacification of the true and false lumina or if the
false channel is very faintly opacified.
79
For these reasons, aortography has generally been replaced by noninvasive imaging
tests in the diagnosis of acute aortic dissection. However, for
patients in whom the suspicion for ascending aortic dissection
is very strong but noninvasive imaging is unavailable or inconclusive, digital subtraction aortography should be performed.
Intravascular ultrasound, in combination with standard aortographic technique, greatly improves the accuracy of aortography,
can be performed rapidly and safely, and could serve as an
accessory diagnostic procedure in selected patients with suspected
aortic dissection.
80,81
In view of the increased early mortality of untreated acute
aortic dissection, the screening test chosen depends on which
test is most readily available at a particular institution and the
patient’s hemodynamic status. Noninvasive diagnosis of acute
aortic dissection by TEE, CT, or MR, if readily available, is

CHAPTER 28 Acute Aortic Syndromes: Diagnosis and Management 297
Intimal ulcer
hematoma
false aneurysm
rupture
(saccular true aneurysm)
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Atheroma
Intima
Media
Adventitia
Plaque ulceration
Intimal deep ulcer
Fig. 28.6 Pathologic consequences of penetrating atherosclerotic ulcer of the aorta. Atheromatous
ulceration that burrows deeply into an atheroma can result in one of four potential outcomes:
true saccular aneurysm, medial hematoma, adventitial false aneurysm, or transmural rupture. An
intramural (medial) hematoma is the most commonly observed consequence of a penetrating
aortic ulcer.
Medial
preferred as it avoids the risks and delays inherent in invasive
angiography.
82
Management
Treatment for acute aortic dissection is initiated when the
diagnosis is first suspected clinically. After initial patient stabilization, the diagnosis is pursued with expedited noninvasive imaging
(Fig. 28.6). Treatment recommendations (discussed later) are
generally in agreement with multidisciplinary cardiovascular
guidelines.
The initial treatment objectives in suspected aortic dissection
are to control pain and provide anti-impulse therapy to reduce
systemic blood pressure, lower the rate of change of pressure
development (dP/dT) to decrease aortic shear stress, and limit
propagation of the dissection. In hypertensive patients, treatment
consists of an intravenous (IV) β-adrenergic blocking agent,
often in combination with IV sodium nitroprusside. β-Blockade
before nitroprusside therapy is essential because the latter increases
the velocity of left ventricular ejection, increases aortic shear
stress, and may promote propagation of the dissection.
venous labetalol in place of a β-blocker and sodium nitroprusside
is an alternative, but it has the potential for hepatotoxicity with
long-term therapy.87 In normotensive patients, an IV β-adrenergic
blocking agent may be used alone. Intravenous verapamil or
diltiazem is an alternative in patients who cannot tolerate
β-blockers. To assist in monitoring blood pressure and renal
perfusion, an intraarterial cannula and indwelling bladder catheter
are advisable. Following initial patient stabilization, expedited
noninvasive aortic imaging is pursued (as discussed in the previous
section). If the patient continues to require close monitoring
and support, an emergency bedside TEE is the test of choice.
Patients with acute type A aortic dissection should undergo
emergent surgical repair, unless significant comorbidities that
limit survival to 1 year or less are present.
graphy before surgery is not indicated because it does not
83–85
8,88,89
Coronary angio-
86
Intra-
Adventitial
Transmural
improve survival and it results in a delay in surgical interven-
90,91
tion.
Dacron is the usual procedure in type A aortic dissection.
Resection of the ascending aorta and replacement with
88,89
The objectives of surgery include excision of the intimal tear,
obliteration of the entrance into the false lumen, repair of aortic
regurgitation (if present), and restoration of patency to any major
arteries occluded by the dissection. With associated aortic
regurgitation, resuspension of the valve, if possible, is preferred.
Intraoperative TEE can define the severity and mechanisms of
aortic regurgitation and can assist the surgeon in identifying
patients in whom valve repair is likely to be successful.92 If there
is associated annuloaortic ectasia or destruction of the aortic
wall, a valved conduit may be used. The coronary arteries are
reimplanted or, if they are involved by the dissection, they are
reattached using vein bypass grafts anastomosed proximally to
the aortic graft and distally to the uninvolved coronary arter-
89,93,94
ies.
If compromise of a branch of the aorta supplying a
vital organ is present and is not relieved by closing the false
channel, then direct repair or surgical replacement of that branch
is indicated. Aortic fenestration may be indicated in patients
with severe organ or limb ischemia complicating either acute
or chronic aortic dissection. Fenestration, by either surgical or
percutaneous techniques, can effectively relieve the ischemia and
can be performed safely in chronic aortic dissection.
95,96
Operative
mortality for type A dissection at experienced centers varies
from 7% to 36%, which is well below the more than 50% mortality
with medical therapy.
7,56,97,98
In-hospital mortality is approximately
14% to 27%, patients with cardiogenic shock and those requiring
concomitant coronary artery bypass grafting being the highest-risk
subgroup.
97,98
There is limited experience with endovascular stent grafts in
patients with type A dissection, although preliminary studies
suggest that endovascular repair can be performed with minimal
adverse effects on aortic valve function and sustained survival.99
Larger studies are needed to confirm the durability of endovascular

298 PART IV Noncoronary Diseases: Diagnosis and Management
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therapy and to more accurately identify those patients most likely
to benefit from this approach.
With acute type B (type III) aortic dissection, urgent surgical
intervention is reserved for patients who have a complicated
course (rupture, acute expansion or impending rupture, or
vascular occlusion).
100
Independent predictors of surgical mortality
include those 70 years and older, and hypotension or stroke on
admission.
101
The optimal treatment for uncomplicated type III
aortic dissection is less well defined, but the majority of patients
are best treated with medical therapy.
mortality for these patients is approximately 10%.
102
Overall in-hospital
103
Long-term
medical therapy emphasizes control of blood pressure, using a
β-blocker if possible, and periodic evaluation for evidence of
any progression of dissection, patency of the false lumen, progression of aortic diameter to greater than 5 cm, or development of
saccular aneurysm.
104–106
The reported long-term survival rate
with medical therapy is approximately 60% to 80% at 4 to 5
years and approximately 40% to 45% at 10 years.
103
Survival is
best in patients with noncommunicating and retrograde dissections. The proximal descending thoracic aorta is the major site
of aneurysm development; an enlarged false lumen in this region
predicts poor outcome and subsequent aneurysm development.
107
Furthermore, partial false lumen thrombosis (34% of patients)
predicts a significantly worse 3-year mortality rate.
108
An alternate
approach is 2 to 3 weeks of pharmacologic therapy followed by
surgical repair if the dissection becomes stable and the patient’s
general condition does not contraindicate surgery.
109,110
Endovascular intervention in type B aortic dissection is being successfully performed with increasing frequency and may provide
an alternative to surgery in highly selected patients in the
111–113
future.
Randomized trials comparing endovascular intervention and conventional medical management have not demonstrated a significant difference in overall survival at 2 years,
although remodeling with thrombosis of the false lumen and
reduction of its diameter was evident in the stent graft group.
114–116
Postoperatively, continuation of β-blockade is essential, if
possible, as hypertension and left ventricular ejection velocity
play an important role in the recurrence of aortic dissection.
Following hospital dismissal, noninvasive testing is performed
at periodic intervals to detect the development of an anastomotic
aneurysm or saccular aneurysm, extension of the dissection,
patency of the false lumen, or progressive aortic dilation.
117,118
Initial follow-up noninvasive imaging at 3, 6, and 12 months is
warranted. Subsequent follow-up is performed every 1 to 2 years
if there is no evidence of disease progression. MRI is generally
preferred for follow-up (a baseline MRI before hospital discharge
should be performed).
PENETRATING AORTIC ULCER
Penetrating aortic ulcer shares several clinical features with
aortic dissection, especially type B aortic dissection, but the
absence of certain clinical signs favors a diagnosis of penetrating
aortic ulcer. Results of noninvasive imaging studies are usually
diagnostic, allowing differentiation of penetrating aortic ulcer
from typical aortic dissection.
two disorders is important in view of the fact that the natural
119,120
Differentiation between the
history of penetrating aortic ulcer is less well defined; therefore,
treatment may differ from that currently used for classic aortic
dissection.
119,120
Pathogenesis
Penetrating aortic ulcer refers to an atherosclerotic lesion of the
thoracic aorta that undergoes ulceration, which penetrates the
internal elastic lamina of the thoracic aorta, resulting in formation
of one of the following: intramural hematoma within the media
of the aortic wall, a true saccular aneurysm, a pseudoaneurysm,
or transmural aortic rupture
121
(Video 28.3).
Predisposing Factors
Risk factors for penetrating aortic ulcer are similar to those for
aortic dissection, the most common being advanced age, chronic
systemic hypertension, and evidence of advanced atherosclerotic
disease.
122
In contrast to aortic dissection, men and women are
equally affected. Long-standing hypertension is present in the
majority of patients and likely contributes to the advanced
atherosclerotic disease that is universally evident. More than
one-half of the patients with penetrating aortic ulcer have
advanced atherosclerotic disease in other locations, including
coronary artery disease, peripheral arterial occlusive disease, and
cerebrovascular disease. An increased association of penetrating
aortic ulcer and abdominal aortic aneurysm, and aneurysms in
other locations, has also been reported.
121,123
Clinical Features
The clinical presentation of penetrating aortic ulcer and acute
aortic dissection is similar, the most common presentation being
an elderly patient with systemic hypertension and the sudden
onset of severe pain in the chest, back, and—less commonly—
epigastrium. Unlike aortic dissection, the pain is rarely migratory.
Since the most common site of penetrating aortic ulcer is in the
descending thoracic aorta, a new murmur of aortic regurgitation,
pericardial friction rub, and peripheral pulse deficits are not
seen. In addition, visceral vessel involvement has not been
reported. Neurologic deficits are very rare, but acute lower
extremity paraplegia may occur.
121
In a patient with a history
compatible with aortic dissection, it is the absence of physical
findings that suggests the diagnosis of penetrating aortic ulcer.
Asymptomatic penetrating aortic ulcer does occur and is usually
incidentally discovered as enlargement of the descending thoracic
aorta or a hilar mass on routine chest radiography or on CT
done for another indication.
121,123
Laboratory Findings
Routine laboratory studies are nonspecific with penetrating
aortic ulcer. Chest radiography is the most helpful of the
routine laboratory tests because it is often abnormal. It may
demonstrate mediastinal widening, focal or diffuse enlargement
of the descending thoracic aorta, a hilar mass, left apical mass,
bilateral pleural effusion, or isolated left pleural effusion.
However, normal chest radiographic findings do not exclude
penetrating aortic ulcer. The most common electrocardiographic
abnormality is left ventricular hypertrophy from chronic systemic
hypertension.
119,120
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