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ACC/AHA Pocket Guideline
Based on the 2010 ACCF/AHA/AATS/
ACR/ASA/SCA/SCAI/SIR/STS/SVM
Guidelines for
the Diagnosis
and Management
of Patients With
Thoracic Aortic
Disease
March 2010
i
Guidelines for
the Diagnosis
and Management
of Patients With
Thoracic Aortic Disease
March 2010
Writing Committee
Loren F. Hiratzka, MD, Chair
George L. Bakris, MD
Joshua A. Beckman, MD, MS
Robert M. Bersin, MPH, MD
Vincent F. Carr, DO
Donald E. Casey, Jr, MD, MPH, MBA
Kim A. Eagle, MD
Luke K. Hermann, MD
Eric M. Isselbacher, MD
Ella A. Kazerooni, MD, MS
Nicholas T. Kouchoukos, MD
Bruce W. Lytle, MD
Dianna M. Milewicz, MD, PhD
David L. Reich, MD
Souvik Sen, MD, MS
Julie A. Shinn, RN, MA, CCRN
Lars G. Svensson, MD, PhD
David M. Williams, MD
© 2010 American College of Cardiology Foundation and
American Heart Association, Inc.
The following material was adapted from the 2010 ACCF/AHA/
AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the
Diagnosis and Management of Patients With Thoracic Aortic
Disease: Executive Summary (Circulation 2010;121:1544–79). This
pocket guideline is available on the World Wide Web sites of the
American College of Cardiology (www.acc.org) and the American
Heart Association (my.americanheart.org).
For copies of this document, please contact Wolters Kluwer Health
Medical Research, Lippincott Williams & Wilkins, email:
Reprintsolutions@wolterskluwer.com; Tel: 410.528.4121; Fax:
410.528.4264.
Permissions: Multiple copies, modification, alteration,
enhancement, and/or distribution of this document are not
permitted without the express permission of the American Heart
Association. Instructions for obtaining permission are located at
www.americanheart.org/presenter.jhtml?identifier=4431. A link to the
“Permission Request Form” appears on the right side of the page.
Contents
1. Introduction
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6
2. Critical Issues
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
10
3. Recommendations for Aortic Imaging Techniques to Determine
the Presence and Progression of Thoracic Aortic Disease
. . . . . . .
14
4. Recommendations for Genetic Syndromes
. . . . . . . . . . . . . . . . . . . .
18
5. Recommendations for Familial Thoracic Aortic Aneurysms and
Dissections
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24
6. Recommendations for Bicuspid Aortic Valve and Associated
Congenital Variants in Adults
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
26
7. Recommendations for Estimation of Pretest Risk of Thoracic
Aortic Dissection
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27
8. Initial Evaluation and Management of Acute Thoracic Aortic
Disease
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
34
8.1. Recommendations for Screening Tests
. . . . . . . . . . . . . . . . . .
34
8.2. Recommendations for Diagnostic Imaging Studies
. . . . . .
35
8.3. Recommendations for Initial Management
. . . . . . . . . . . . . .
36
8.4. Recommendations for Definitive Management
. . . . . . . . . .
37
9. Recommendation for Surgical Intervention for Acute Thoracic
Aortic Dissection
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
41
2
3
10. Recommendation for Intramural Hematoma Without Intimal
Defect
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
42
11. Recommendation for History and Physical Examination for
Thoracic Aortic Disease
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
43
12. Recommendation for Medical Treatment of Patients With
Thoracic Aortic Diseases
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
44
12.1. Recommendations for Blood Pressure Control
. . . . . . . . . . .
44
13. Recommendations for Asymptomatic Patients With Ascending
Aortic Aneurysm
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
46
14. Recommendation for Symptomatic Patients With Thoracic
Aortic Aneurysm
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
50
15. Recommendations for Open Surgery for Ascending Aortic
Aneurysm
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
51
16. Recommendations for Aortic Arch Aneurysms
. . . . . . . . . . . . . . . . .
52
17. Recommendations for Descending Thoracic Aorta and
Thoracoabdominal Aortic Aneurysms
. . . . . . . . . . . . . . . . . . . . . . . . .
54
18. Recommendations for Counseling and Management of
Chronic Aortic Diseases in Pregnancy
. . . . . . . . . . . . . . . . . . . . . . . . .
56
19. Recommendations for Aortic Arch and Thoracic Aortic
Atheroma and Atheroembolic Disease
. . . . . . . . . . . . . . . . . . . . . . . .
58
4
5
20. Periprocedural and Perioperative Management
. . . . . . . . . . . . . . . .
59
20.1. Recommendations for Brain Protection During Ascending
Aortic and Transverse Aortic Arch Surgery
. . . . . . . . . .
59
20.2. Recommendations for Spinal Cord Protection During
Descending Aortic Open Surgical and Endovascular
Repairs
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
60
21. Recommendations for Surveillance of Thoracic Aortic
Disease or Previously Repaired Patients
. . . . . . . . . . . . . . . . . . . . . .
62
22. Recommendation for Employment and Lifestyle in
Patients With Thoracic Aortic Disease
. . . . . . . . . . . . . . . . . . . . . . . . .
64
6
1. Introduction
The term “thoracic aortic disease” encompasses a
broad range of degenerative, structural, acquired,
genetic-based, and traumatic disease states and pre-
sentations. According to the Centers for Disease
Control and Prevention death certificate data, dis-
eases of the aorta and its branches account for 43
000 to 47 000 deaths annually in the United States.
The precise number of deaths attributable to thorac-
ic aortic diseases is unclear. However, autopsy stud-
ies suggest that the presentation of thoracic aortic
disease is often death due to aortic dissection (AoD)
and rupture, and these deaths account for twice as
7
many deaths as attributed to ruptured abdominal
aortic aneurysms (AAAs). This guideline includes
diseases involving any or all parts of the thoracic
aorta with the exception of aortic valve diseases and
includes the abdominal aorta when contiguous tho-
racic aortic diseases are present.
8
Table 1.
Applying Classification of
Recommendations and Level of Evidence
LeveL A
Multiple populations
evaluated*
Data derived from multi-
ple randomized clinical
trials or meta-analyses
LeveL B
Limited populations
evaluated*
Data derived from a
single randomized trial or
nonrandomized studies
LeveL C
Very limited populations
evaluated*
Only consensus opinion
of experts, case studies,
or standard of care
CLASS I
Benefit >>> Risk
Procedure/Treatment
shOuLD be performed/
administered
n
Recommendation that
procedure or treatment
is useful/effective
n
sufficient evidence from
multiple randomized trials
or meta-analyses
n
Recommendation that
procedure or treatment
is useful/effective
n
Limited evidence from
single randomized trial or
nonrandomized studies
n
Recommendation that
procedure or treatment is
useful/effective
n
Only expert opinion, case
studies, or standard of care
CLASS IIA
Benefit >> Risk
Additional studies with
focused objectives needed
IT Is ReasOnabLe to per-
form procedure/administer
treatment
n
Recommendation in favor
of treatment or procedure
being useful/effective
n
some conflicting evidence
from multiple randomized
trials or meta-analyses
n
Recommendation in favor
of treatment or procedure
being useful/effective
n
some conflicting evidence
from single randomized trial
or nonrandomized studies
n
Recommendation in favor
of treatment or procedure
being useful/effective
n
Only diverging expert
opinion, case studies,
or standard of care
should
is recommended
is indicated
is useful/effective/beneficial
suggested phrases for
writing recommendations
†
is reasonable
can be useful/effective/beneficial
is probably recommended
or indicated
S I z E O F T R E A T M E n T E F F E C T
E
STIMA
TE
o
F
C
ERT
AI
n
T
y (P
RECISI
on) o
F
T
REA
TME
n
T
E
FFECT
9
Class IIb
Benefit
≥
Risk
Additional studies with broad
objectives needed; additional
registry data would be helpful
Procedure/Treatment
May be cOnsIDeReD
n
Recommendation’s
usefulness/efficacy less
well established
n
Greater conflicting
evidence from multiple
randomized trials or
meta-analyses
n
Recommendation’s
usefulness/efficacy less
well established
n
Greater conflicting
evidence from single
randomized trial or
nonrandomized studies
n
Recommendation’s
usefulness/efficacy less
well established
n
Only diverging expert
opinion, case studies, or
standard of care
Class III
Risk
≥
Benefit
Procedure/Treatment should
nOT be performed/adminis-
tered sInce IT Is nOT heLP-
fuL anD May be haRMfuL
n
Recommendation that
procedure or treatment is
not useful/effective and
may be harmful
n
sufficient evidence from
multiple randomized trials
or meta-analyses
n
Recommendation that
procedure or treatment is
not useful/effective and
may be harmful
n
Limited evidence from
single randomized trial or
nonrandomized studies
n
Recommendation that
procedure or treatment is
not useful/effective and
may be harmful
n
Only expert opinion, case
studies, or standard of care
may/might be considered
may/might be reasonable
usefulness/effectiveness is
unknown /unclear/uncertain
or not well established
is not recommended
is not indicated
should not
is not useful/effective/beneficial
may be harmful
* Data available from clinical trials
or registries about the usefulness/
efficacy in different
subpopulations, such as sex, age,
history of diabetes, history of
prior myocardial infarction, history
of heart failure, and prior aspirin
use. A recommendation with
Level of Evidence B or C does not
imply that the recommendation is
weak. Many important clinical
questions addressed in the
guidelines do not lend themselves
to clinical trials. Even though
randomized trials are not available,
there may be a very clear clinical
consensus that a particular test or
therapy is useful or effective.
† In 2003, the ACCF/AHA Task Force
on Practice Guidelines developed
a list of suggested phrases to use
when writing recommendations.
All guideline recommendations
have been written in full
sentences that express a
complete thought, such that a
recommendation, even if
separated and presented apart
from the rest of the document
(including headings above sets of
recommendations), would still
convey the full intent of the
recommendation. It is hoped that
this will increase readers’
comprehension of the guidelines
and will allow queries at the
individual recommendation level.
10
2. Critical Issues
As the writing committee developed this guideline, several criti-
cal issues emerged:
n
Thoracic aortic diseases are usually asymptomatic and
not easily detectable until an acute and often catastrophic
complication occurs. Imaging of the thoracic aorta with
computed tomographic imaging (CT), magnetic resonance
imaging (MR), or in some cases, echocardiographic
examination is the only method to detect thoracic aortic
diseases
n
Radiologic imaging technologies have improved in terms
of accuracy of detection of thoracic aortic disease.
However risks associated with repeated radiation
exposure, as well as contrast medium–related toxicity have
also been recognized. The writing committee therefore
formulated recommendations on a standard reporting
format (Section 3) as well as surveillance schedules
(Section 21).
n
Imaging for asymptomatic patients at high risk based on
history or associated disease is expensive and not always
covered by payers.
n
For many thoracic aortic diseases, results of treatment for
stable, often asymptomatic, but high-risk conditions are far
better than the results of treatment required for acute and
often catastrophic disease presentations. Thus, the
identification and treatment of patients at risk for acute and
11
catastrophic disease presentations (eg, thoracic AoD and
thoracic aneurysm rupture) prior to such an occurrence are
paramount to eliminating the high morbidity and mortality
associated with acute presentations.
n
Patients with acute AoD are subject to missed or delayed
detection of this catastrophic disease state. Many present
with atypical symptoms and findings, making diagnosis
even more difficult. Awareness of the varied and complex
nature of thoracic aortic disease presentations has been
lacking, especially for acute AoD. Risk factors and clinical
presentation clues are noted in Section 7. The
collaboration of multiple medical specialties for this
guideline will provide opportunities to raise the level of
awareness among all medical specialties.
n
There is rapidly accumulating evidence that genetic
alterations or mutations predispose some individuals to
aortic diseases (see Sections 4-6). Therefore, identification
of the genetic alterations leading to these aortic diseases
has the potential for early identification of individuals at
risk. In addition, biochemical abnormalities involved in the
progression of aortic disease are being identified through
studies of patients’ aortic samples and animal models of
the disease. The biochemical alterations identified in the
aortic tissue have the potential to serve as biomarkers for
aortic disease. Understanding the molecular pathogenesis
may lead to targeted therapy to prevent aortic disease.
Medical and gene-based treatments are beginning to show
promise for reducing or delaying catastrophic
complications of thoracic aortic diseases.
12
Figure 1.
normal Anatomy of the Thoracoabdominal Aorta.
13
Anatomic Location
1.
Aortic sinuses of Valsalva
2.
Sinotubular junction
3.
Mid ascending aorta (midpoint in length between Nos. 2 and 4)
4.
Proximal aortic arch (aorta at the origin of the innominate
artery)
5.
Mid aortic arch (between left common carotid and subclavian
arteries)
6.
Proximal descending thoracic aorta (begins at the isthmus,
approximately 2 cm distal to left subclavian artery)
7.
Mid descending aorta (midpoint in length between Nos. 6 and
8)
8.
Aorta at diaphragm (2 cm above the celiac axis origin)
9.
Abdominal aorta at the celiac axis origin
Normal anatomy of the thoracoabdominal aorta with standard anatomic landmarks for reporting aortic
diameter as illustrated on a volume-rendered CT image of the thoracic aorta. CT indicates computed
tomographic imaging.
14
3. Recommendations for Aortic Imaging
Techniques to Determine the Presence and
Progression of Thoracic Aortic Disease
Class I
1.
Measurements of aortic diameter should be taken
at reproducible anatomic landmarks, perpendicular
to the axis of blood flow, and reported in a clear
and consistent format. (LOE: C)
2.
For measurements taken by computed
tomographic imaging or magnetic resonance
imaging, the external diameter should be measured
perpendicular to the axis of blood flow. For aortic
root measurements, the widest diameter, typically at
the mid-sinus level, should be used. (LOE: C)
3.
For measurements taken by echocardiography, the
internal diameter should be measured perpendicular
to the axis of blood flow. For aortic root
measurements the widest diameter, typically at the
mid-sinus level, should be used. (LOE: C)
4.
Abnormalities of aortic morphology should be
recognized and reported separately even when
aortic diameters are within normal limits. (LOE: C)
15
5.
The finding of aortic dissection, aneurysm,
traumatic injury and/or aortic rupture should be
immediately communicated to the referring
physician. (LOE: C)
6.
Techniques to minimize episodic and cumulative
radiation exposure should be utilized whenever
possible. (LOE: B)
Class IIa
1.
If clinical information is available, it can be useful
to relate aortic diameter to the patient’s age and
body size. (LOE: C)
16
Table 2.
Essential Elements of Aortic Imaging Reports
1. The location at which the aorta is abnormal.
2. The maximum diameter of any dilatation, measured from the
external wall of the aorta, perpendicular to the axis of flow, and the
length of the aorta that is abnormal.
3. For patients with presumed or documented genetic syndromes at
risk for aortic root disease measurements of aortic valve, sinuses of
Valsalva, sinotubular junction, and ascending aorta.
4. The presence of internal filling defects consistent with thrombus
or atheroma.
5. The presence of IMH, PAU, and calcification.
6. Extension of aortic abnormality into branch vessels, including
dissection and aneurysm, and secondary evidence of end-organ
injury (eg, renal or bowel hypoperfusion
7. Evidence of aortic rupture, including periaortic and mediastinal
hematoma, pericardial and pleural fluid, and contrast extravasation
from the aortic lumen.
8. When a prior examination is available, direct image to image
comparison to determine if there has been any increase in diameter.
IMH indicates intramural hematoma; and PAU, penetrating atherosclerotic ulcer.
17
Table 3.
normal Adult Thoracic Aortic Diameters
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