Guidelines for Diagnosis and Treatment of Aortic Aneurysm and Aortic Dissection

40 Pages·2013·1.45 MB·English

Circulation Journal JCS GUIDELINES
Official Journal of the Japanese Circulation Society
http://www.j-circ.or.jp
Guidelines for Diagnosis and Treatment of Aortic
Aneurysm and Aortic Dissection (JCS 2011)
– Digest Version –
JCS Joint Working Group
Table of Contents
Introduction of the Revised Guidelines ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙789 2. Thoracic Aortic Aneurysm ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙804
I D efinition/Classification and Pathology/ 3. Abdominal Aortic Aneurysm ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙805
Epidemiology ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙790 V Surgical Treatment ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙805
1. Definition ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙790 1. Thoracic Aorta ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙805
2. Glossary∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙791 2. Abdominal Aorta ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙808
3. Classifications and Pathological Conditions ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙792 VI Endovascular Treatment ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙809
4. Statistics and Epidemiology ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙794 1. Aortic Dissection (Recommendations for
II Diagnosis ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙795 Endovascular Treatment of Aortic Dissection) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙809
1. General Remarks ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙795 2. Thoracic Aortic Aneurysm (Recommendations for
2. Radiological Diagnosis: Plain X-Ray Examination, Stent-Graft Treatment of Thoracic Aortic Aneurysm) ∙∙∙∙809
CT, Angiography ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙795 3. Abdominal Aortic Aneurysm (Recommendations for
3. Ultrasonography ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙797 Stent-Graft Treatment for Abdominal Aortic
4. MRI ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙798 Aneurysm) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙813
5. Identification of Adamkiewicz Artery ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙798 VII Specific Pathological Conditions ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙816
III Choice of Treatment Methods ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙798 1. Marfan Syndrome ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙816
1. Aortic Dissection (Recommendations for Choosing 2. Inflammatory Abdominal Aortic Aneurysm ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙817
Treatment Methods for Acute Aortic Dissection) ∙∙∙∙∙∙∙∙∙∙∙798 3. Infective Aortic Aneurysm ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙818
2. Thoracic Aortic Aneurysm (Recommendations for VIII Aortic Disease and Genetics ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙818
Choosing Treatment Methods for Thoracic Aortic 1. Aortic Disease and Genetics ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙818
Aneurysm) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙801 2. Genetic Test ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙818
3. Abdominal Aortic Aneurysm (Recommendations for 3. Disease Specifics ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙818
Choosing Treatment Methods for Abdominal Aortic References ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙820
Aneurysm) ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙801
(Circ J 2013; 77: 789 – 828)
IV Medical Treatment ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙802
1. Aortic Dissection ∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙∙802
Introduction of the Revised Guidelines
Since the “Guidelines for Diagnosis and Treatment of Aortic of the descending aorta. In addition, much about the relation-
Aneurysm and Aortic Dissection (JCS 2006)” were issued by ship between aortic diseases and genetic abnormalities has
the Japanese Circulation Society (JCS) in 2006,1 we have seen become apparent, and new aspects of treatment have evolved.
advances in the treatment of aortic diseases, and partial revi- Chapters that address these new aspects of treatment in the
sion of the Guidelines has thus been approved by the JCS present guidelines provide particularly detailed descriptions.
Scientific Committee. Over the past 5 years, stent-grafting has These guidelines also indicate the difference between the in-
rapidly become common in the treatment of aortic diseases in terpretation of aortic dissection in Japan and that in Western
Japan, being employed in many institutions and becoming in- countries. Accordingly, this update of the guidelines represents,
dispensable in the management of aortic diseases, particularly in practical terms, a full-scale revision of the guidelines.
Released online February 13, 2013
Mailing address: Scientific Committee of the Japanese Circulation Society, 8th Floor CUBE OIKE Bldg., 599 Bano-cho, Karasuma
Aneyakoji, Nakagyo-ku, Kyoto 604-8172, Japan. E-mail: [email protected]
This English language document is a revised digest version of Guidelines for Diagnosis and Treatment of Aortic Aneurysm and Aortic
Dissection reported at the Japanese Circulation Society Joint Working Groups performed in 2010 (website: http://www.j-circ.or.jp/
guideline/pdf/JCS2011_takamoto_d.pdf).
Joint Working Groups: The Japanese Circulation Society, The Japan Radiological Society, The Japanese Association for Thoracic Surgery,
The Japanese Society for Vascular Surgery, The Japanese Society for Cardiovascular Surgery, The Japanese College of Cardiology, The
Japanese College of Angiology
ISSN-1346-9843 doi: 10.1253/circj.CJ-66-0057
All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: [email protected]
Circulation Journal Vol.77, March 2013
790 JCS Joint Working Group
The incidence of aortic diseases is high in Japan in com- We expect that these guidelines will serve as a good treat-
parison with other countries worldwide. In particular, the fre- ment guide for aortic dissection and aortic aneurysm. However,
quency of aortic dissection is ranked at the top, paralleling the guidelines are only indices that are based on the currently avail-
situation in Italy. The cited underlying factors include a high able evidence, and are not obligatory rules that require com-
prevalence of hypertension, a large proportion of elderly in the plete adherence. It should be recognized that physicians who
population, and high availability of computed tomography (CT) are particularly specialized in this field may choose a certain
which facilitates the diagnosis of aortic diseases. Intramural treatment that deviates from the guidelines, based on sufficient
hematoma (IMH) is a type of aortic dissection commonly re- knowledge of the evidence from research and practical pro-
ferred to in Western countries. The pathological condition of cesses of new treatment as well as better medical services.
this disease is, essentially, hemorrhage in the tunica media of However, it is important for young physicians to fully under-
the aortic wall, resulting in hematoma. There is currently dis- stand these guidelines in order to attain an up-to-date level of
cussion about the relationship between this condition and clas- medical practice.
sic aortic dissection that originates from a tear in the tunica Japan has a high frequency of aortic diseases in comparison
intima, with dissection progressing in the tunica media. Fun- with other countries in the world, and is enjoying a high level
damentally, IMH is a pathological diagnosis. However, the of accuracy and much better results in medical practice which
current situation is that radiologists make a diagnosis of IMH surpass those in Western countries. On behalf of all persons
only because there is no evidence of the presence of a tear on involved in the revision of these guidelines, we sincerely hope
diagnostic imaging, or because the dissected lumen is not vi- that these guidelines lead to further improvements in the level
sualized. According to the American College of Cardiology of healthcare in Japan and to saving more patients and achiev-
Foudation/American Heart Association (ACCF/AHA) guide- ing greater quality of life (QOL) for those affected by aortic
lines for the diagnosis and management of patients with Tho- diseases.
racic Aortic Disease issued in 2010,2 a theoretically incongru- The following classifications are used for expressing the
ous concept of IMH with ulcer-like projection (ULP) has been class of recommendations for particular methods of diagnosis
presented. In the Western countries, a diagnosis of IMH is and treatment and the level of evidence according to the rele-
often made by a single CT session in clinical practice, and it vant American College of Cardiology (ACC)/AHA Guide-
is explained that a tear may form after a diagnosis of IMH and lines (http://circ.ahajournals.org/manual/manual_IIstep6.shtml;
result in communicating aortic dissection. However, it is not available in January 2011).
certain that this explanation is valid. It is possible that the con-
trast agent does not enter the dissected lumen because of blood Classification of Recommendations
retention in the dissected lumen without reentry despite a tear Class I: Conditions for which there is evidence and/or general
being present from the beginning. In Western countries, cases agreement that a given procedure or treatment is use-
with thrombotic occlusion of the false lumen of the ascending ful and effective.
aorta in type III retrograde dissection have actually been re- Class II: Conditions for which there is conflicting evidence and/
ferred to as type-A IMH. In addition, in Western countries, the or a divergence of opinion about the usefulness/effi-
efficacy of medical treatment for IMH is considered to be poor cacy of a procedure or treatment.
because IMH may proceed to classic aortic dissection in the IIa: Weight of evidence/opinion is in favor of useful-
future. In contrast, in Japan and South Korea, the frequent use ness/efficacy.
of CT allows an accurate understanding of changes in the IIb: Usefulness/efficacy is less well established by evi-
pathological features of this condition, and observation of the dence/opinion.
disease course upon medical treatment thus generally achieves Class III: Conditions for which there is evidence and/or gen-
favorable results. eral agreement that the procedure/treatment is not
Therefore, this set of guidelines provides the view that the useful/effective, and in some cases may be harmful.
term IMH, which may lead to an incorrect understanding of
the pathological condition, should not be used clinically in Level of Evidence
Japan. We believe that non-communicating aortic dissection Level of Evidence: A
is the proper term that accurately expresses the pathological Data derived from multiple randomized clinical trials
features of this condition, and is more useful for deciding an Level of Evidence: B
appropriate therapeutic strategy in clinical practice. Discussion Data derived from a single randomized trial, or non-ran-
should be continued through academic meetings in Western domized studies
countries, concerning this discrepancy from ACCF/AHA guide- Level of Evidence: C
lines. Consensus opinion of experts
I Definition/Classification and Pathology/Epidemiology
Although the flap in aortic dissection usually has one to sev-
1. Definition
eral tears, some cases have no clear tear or no communication
between the true lumen and the false lumen. The former type
1. Aortic Dissection is called communicating aortic dissection,4 and the latter non-
Aortic dissection is a condition in which the aortic wall is de- communicating aortic dissection4 (synonymous with the con-
tached into two layers at the medial level, causing two lumens ventional thrombosed type).
having a certain length along the arterial course. This is a dy- Recent progress in diagnostic imaging has allowed us to
namic condition characterized by the presence of blood flow detect a condition in which the aortic media is detached due to
or hematoma in the aortic wall.3,4 hematoma, without an observable tear. This condition is called
Circulation Journal Vol.77, March 2013
JCS Guidelines for Aortic Aneurysm and Aortic Dissection 791
Figure 2. Saccular type aortic aneurysm.
Figure 1. Fusiform type aortic aneurysm.
or saccular, respectively.
2. Glossary
IMH or intramural hemorrhage, representing the distinct path-
ological concept termed “aortic dissection without tear5”. How- Aortic dissection
ever, because IMH is based on a pathological diagnosis, we - Dissecting aneurysm of the aorta: Aortic dissection forming
have decided not to use this term in the clinical setting. So- an aneurysm.
called IMH, devoid of tears on diagnostic imaging, is clini- - C lassic aortic dissection: Dissection with a tear or flap, in
cally regarded as non-communicating aortic dissection (syn- contrast to IMH.
onymous with the conventional thrombosed type) and dealt - T rue lumen: Original arterial lumen.
with as a type of dissection.6 - False lumen: New lumen formed in the wall (dissected
Clinically, it is often difficult to clearly distinguish between lumen is an inappropriate term).
IMH and aortic dissection that has an intimal defect and tear - Flap: Septum (of the internal media). Although also referred
(ULP on diagnostic imaging), without evidence of blood flow to as detached intima, a flap actually consists of part of the
in the false lumen (thrombosed false lumen with intimal de- intima and media.
fect, ULP-type). In addition, because the ability to detect ULP - T ear: A tear involving parts of the intima and media and
varies among different diagnostic imaging techniques, and representing a communication between the true lumen and
ULP-type dissection includes some unstable cases regardless false lumen in dissection. Intimal tear is also idiomatically
of the size of the ULP, this condition is clinically important. used as a synonym.
To bring clinical attention to the importance of ULP, it is - Entry: An area where blood flow enters from the true lumen
recommended that ULP-type dissection be dealt with as com- into the false lumen.
municating aortic dissection.6 - R eentry: An area where blood flow enters from the false
In addition, cases with partial thrombus in a false lumen and lumen into the true lumen.
a thrombosed false lumen communicating with the true lumen - Communicating aortic dissection: The same as communicat-
should definitely be classified into the category of communi- ing aortic dissection in the classification by European Soci-
cating aortic dissection. ety of Cardiology (ESC), also known as classic dissection
Although penetrating atherosclerotic ulcer (PAU) was pro- and double barreled aorta.
posed as a concept that represents an ulcerated aortic athero- - N on-communicating aortic dissection: The same as non-
sclerotic lesion that extends to the media,7 much remains un- communicating aortic dissection in the classification by ESC.
clear as to the relationship between PAU and aortic dissection. - T hrombosed type aortic dissection: Synonymous with non-
Various issues about IMH and PAU remain to be clarified, and communicating aortic dissection.
due caution is therefore necessary when applying these terms. - I ntramural hematoma (IMH): Used almost synonymously
with dissection without a tear from the pathological view-
2. Aortic Aneurysm point or non-communicating aortic dissection from the clin-
Aortic aneurysm is a circumferential or local enlargement (in- ical viewpoint. This term is not, however, used clinically be-
creased diameter) or protrusion of a part of the aortic wall. cause it is based on a pathological diagnosis.
When a part of the aortic wall is dilated and thereby forms - I ntramural hemorrhage: Synonymous with IMH.
a bump, or when the diameter is increased to a degree at least - U lcer-like projection (ULP): Small protrusion found in part
1.5-fold greater than normal (exceeding 45 mm in the thoracic of the false lumen on diagnostic imaging including arteriog-
region and 30 mm in the abdominal region) in a fusiform man- raphy. Although detection abilities vary among different di-
ner, the condition is called aneurysm.8,9 agnostic imaging techniques, the findings of ULP on imag-
Aortic aneurysm is a localized dilatation (increased diam- ing include various pathological conditions (e.g., tear, rupture
eter) or protrusion of the aortic wall, and is called a fusiform of a branch, atherosclerotic ulceration). Clinically, ULP is
type aortic aneurysm (Figure 1) or a saccular type aortic an- an unstable condition regardless of the size, such that close
eurysm (Figure 2) according to whether the shape is fusiform observation is necessary. Therefore, to assure clinical atten-
Circulation Journal Vol.77, March 2013
792 JCS Joint Working Group
Table 1. Classification of Aortic Dissection
1. Classification based on the extent of dissection
Stanford classification
Type A: Dissection involving the ascending aorta
Type B: Dissection not involving the ascending aorta
DeBakey classification
Type I: A tear located in the ascending aorta and dissection extending to the aorta distal to the aortic arch
Type II: Dissection confined to the ascending aorta
Type III: A tear located in the descending aorta
Type IIIa: Dissection not involving the abdominal aorta
Type IIIb: Dissection involving the abdominal aorta
The following subcategories can be added to the DeBakey classification
Arch type: A tear located in the aortic arch
Arch localized type: Dissection confined to the aortic arch
Arch extended type: Dissection extending to the ascending or descending aorta
Abdominal type: A tear located in the abdominal aorta
Abdominal localized type: Dissection confined to the abdominal aorta
Abdominal extended type: Dissection extending to the thoracic aorta
(The term “retrograde type III dissection” is not used.)
2. Classification based on blood flow in the false lumen
Communicating type: Presence of blood flow in the false lumen. Also included in this category are partially thrombosed false lumen or
cases with blood flow in the long axis direction in the false lumen from the ULP, despite a large portion of the false
lumen being thrombosed.
ULP-type: No blood flow in a large portion of the false lumen, but blood flow localized near the tear (ULP)
Non-communicating type: Crescent-shaped false lumen without either tear (including ULP) or blood flow in the false lumen
3. Classification based on the disease phase
Acute phase: Presentation within 2 weeks following the initial event. Very acute phase refers to those presenting within the first 48 hours.
Chronic phase: Presentation at 2 weeks or longer following the initial event
ULP, ulcer-like projection.
tion, it is recommended that dissection with ULP be treated refers to the part from the level of the 3rd to 4th thoracic
in the same manner as communicating aortic dissection. vertebra and below.
- R e-dissection: New dissection occurring at a site other than - Thoracoabdominal aortic aneurysm (TAAA): The name
the initial dissection. given to an aneurysm involving a continuous area from the
- R e-canalization: A condition where blood flow enters the thorax to the abdominal cavity. TAAA is divided into 4 types
closed false lumen formerly without blood flow in cases with according to Crawford classification.
non-communicating aortic dissection or with closure of the - A bdominal aortic aneurysm (AAA): The name given to an
false lumen in communicating aortic dissection. aneurysm occurring in the abdominal aorta.
- E xtension of dissection: Extension of dissection mainly in - I nflammatory abdominal aortic aneurysm (IAAA).
the long axis direction. If a dissection once terminated ex- - T rue aneurysm of the aorta: Synonymous with so-called aor-
tends again after a certain period of time, it may be classified tic aneurysm. This term is used when clear distinction from
into the re-dissection category. pseudoaneurysm of the aorta is intended. Although the wall
- Enlargement of dissection (false lumen): Enlargement of the of the aneurysm consists of the original arterial wall, the
false lumen mainly in the short axis direction. media may not be histologically confirmed when the aneu-
Aortic aneurysm rysm is enlarged.
- F usiform type aortic aneurysm. - P seudoaneurysm (false aneurysm) of the aorta: Aneurysm
- S accular type aortic aneurysm. having no aortic wall structure. This type of aneurysm often
Fusiform type and saccular type aneurysms: Circumferential has a traumatic or infectious etiology.
enlargement of the aortic wall to a diameter 1.5-fold greater
than normal is called fusiform type aneurysm, whereas knob-
3. Classifications and Pathological Conditions
by protrusion of part of the aortic wall is called saccular type
aneurysm. If there is no clear differentiation, the case should
be dealt with as a saccular type lesion. 1. Aortic Dissection
- T horacic aortic aneurysm (TAA): The name given to an (1) Classifications
aneurysm occurring in the aorta of the thorax. Ascending Table 1 shows 1) classification by the extent of dissection, 2)
aorta refers to the part beginning from the aortic annulus to classification by status of blood flow in the false lumen, and 3)
the bifurcation of the brachiocephalic artery, aortic arch re- classification by disease phase.
fers to the origin of the brachiocephalic artery to the level of
the 3rd to 4th thoracic vertebra (the sites of right and left (2) Pathological Conditions
bifurcations of the pulmonary artery) and descending aorta Aortic dissection essentially characterized by dissection of the
Circulation Journal Vol.77, March 2013
JCS Guidelines for Aortic Aneurysm and Aortic Dissection 793
Figure 3. Pathological conditions of aortic
dissection.
aortic wall and blood flow into the dissection is subject to the aortic wall due to rupture of a blood vessel feeding the
changes over time beginning from immediately after onset, aorta.6
and therefore presents as dynamic pathological conditions. Non-communicating aortic dissection is defined as follows:
These pathological conditions are also variable because the
lesion extends to a broad zone of the blood vessel (Figure 3). 1. It has a crescent-shaped false lumen.
The wide spectrum of pathological conditions is better under- 2. There is no tear and blood flow entering from a tear. Name-
stood if the status of the blood vessel is classified into 1) dila- ly, there is no communication between the false lumen and
tation, 2) rupture, and 3) stenosis or occlusion, and combined the true lumen.
with the site of dissection.
If there is obvious blood flow in the long axis direction in
1) Dilatation the false lumen, the lesion should not be dealt with as non-
(a) Aortic valve insufficiency communicating aortic dissection. In these guidelines, ULP-
(b) Aneurysm formation type dissection and non-communicating dissection are defined
2) Rupture as different pathological conditions (Figure 4). On the other
(a) Cardiac tamponade hand, cases with a thrombosed false lumen resulting from
(b) Bleeding into the thoracic cavity or other areas retrograde dissection that arose from a tear in the descending
3) P eripheral Circulatory Disturbance Due to Stenosis thoracic aorta or abdominal aorta should be classified as com-
or Occlusion of the Aortic Branches municating dissection, although their imaging findings closely
(a) Angina, myocardial infarction resemble with those of non-communicating dissection
(b) Cerebral ischemia (Figure 5).
(c) Upper limb ischemia
(d) Paraplegia 2. Aortic Aneurysm
(e) Intestinal tract ischemia (1) Classification
(f) Renal failure 1) Morphology of the Aneurysm Wall
(g) Lower limb ischemia (a) True aneurysm of the aorta
4) Other Pathological Conditions (b) Pseudoaneurysm of the aorta
(a) Disseminated intravascular coagulation syndrome (c) Dissecting aneurysm of the aorta
(DIC), pre-DIC 2) Location of the Aneurysm
(b) Pleural effusion (a) Thoracic region
(c) Systemic inflammatory response syndrome (b) Thoracoabdominal region
(c) Abdominal region
(3) Definition of Non-Communicating Aortic Dissection 3) Etiology
Because it is difficult to clinically distinguish between dissec- (a) Atherosclerotic
tion without a tear and dissection with a tear showing no blood (b) Traumatic
flow in the false lumen, it is preferable to avoid the use of the (c) Inflammatory
term aortic IMH, which pathologically denotes hematoma in (d) Infective
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794 JCS Joint Working Group
Table 2. Clinical Signs and Symptoms of Aortic Aneurysm
1. Pain
Dissection, rupture
2. Signs and symptoms caused by compression
In thorax: Hoarseness, dysphagia, facial edema
In abdomen: Abdominal distension
3. Sings and symptoms caused by ischemia of organs
Branches of the aortic arch (brain), spinal artery, branches
of the abdominal aorta (e.g., intestinal tract), renal artery,
arteries of the lower limb
Signs and symptoms vary according to the organs perfused
by the arteries
(e) Congenital
4) Shape of the Aneurysm
(a) Fusiform type
Figure 4. ULP-type aortic dissection and non-communicating
(b) Saccular type
aortic dissection. (A) Aortic dissection showing a ULP (arrow).
If the ULP expands along its long axis and dissected flaps are
observed in more than 2 views in the axial CT or MRI imaging, (2) Pathological Conditions
it is classified as communicating dissection (Figure 5C). (B) Signs and symptoms of aortic aneurysm are shown in
Thrombosed false lumen with a tear but without blood flow, Table 2.10
which can be diagnosed during surgery or with MDCT. It is
practically impossible to demonstrate this by imaging tears
without blood flow or with minimum blood flow. These cases, 4. Statistics and Epidemiology
therefore, are classified as non-communicating dissection.
(C) Non-communicating dissection without tears. This refers
to narrowly defined non-communicating dissection and aortic 1. Incidence Rate per Year
intramural hematoma. CT, computed tomography; MDCT, Limited regional investigation data indicate that the incidence
multi-detector computed tomography; MRI, magnetic reso- rate per year seems to be about 3 out of 100,000 population.11
nance imaging; ULP, ulcer-like projection.
According to the statistics reported by the Japanese Asso-
ciation for Thoracic Surgery,12 there were 5,985 cases under-
going surgery for non-dissecting thoracic aortic aneurysm
(TAA) or thoracoabdominal aortic aneurysm (TAAA) and
5,013 cases undergoing surgery for dissecting aortic aneurysm
in 2008. The number of surgical cases tends to be increasing
annually.12–16
2. Changes in the Incidence Rate According to Age
Based on estimations from autopsy cases, the onset of aortic
dissection peaks in both men and women in their 70 s,17 where-
as the peak for non-dissecting aortic aneurysm is in men in
their 70 s and in women in their 80 s.17
3. C hanges in the Incidence Rate According to the
Season, Time, and Day of the Week
The onset of aortic aneurysm tends to be more frequent in win-
ter and less frequent in summer.18–20 In terms of time, aortic
aneurysm occurs more frequently in the daytime, i.e., when
people are active; the onset is reported to be particularly fre-
quent during the period of 6:00 to 12:00. Conversely, the onset
is rare from late at night until early in the morning.18,19,21
Figure 5. Patterns of false lumen in communicating aortic
dissection. (A) Typical communicating dissection. Antegrade
4. Aortic Dissection in Cases of Sudden Death
blood flow observed in the false lumen. (B) Although there is
a tear in the abdominal aorta with blood flow in the false lumen According to a report from the Tokyo Metropolitan Medical
distal to the tear, although the false lumen in the descending Examiner’s Office,20 pre-hospital mortality rate accounted for
thoracic aorta proximal to the tear is mostly thrombosed with 61.4%, death within an hour after onset for 7.3%, 1 to 6 hours
no blood flow. The thoracic aorta is similar to that in non- after onset for 12.4%, and 6 to 24 hours after onset for 11.7%
communicating dissection. (C) Although the false lumen is
of mortalities associated with aortic dissection. Combining
mostly thrombosed, part of the lumen (arrow) demonstrates
pre-hospital with in-hospital mortality rates shows that 93% of
blood flow in the long axis direction. This is classified as com-
municating dissection, which also includes cases that have deaths from aortic dissection occur within 24 hours after
developed from the non-communicating or ULP-type dissec- onset.
tion. ULP, ulcer-like projection.
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JCS Guidelines for Aortic Aneurysm and Aortic Dissection 795
II Diagnosis
termine the most appropriate therapeutic strategy.
1. General Remarks
2. Radiological Diagnosis: Plain X-Ray
1. Acute Aortic Dissection
Examination, CT, Angiography
It is of utmost importance to maintain suspicion when diag-
nosing acute aortic dissection. Diagnosis should be made ac-
cording to the flow chart shown in Figure 6. 1. Plain X-Ray Examination
(1) Aortic Aneurysm
2. True Aortic Aneurysm TAA is often detected by plain chest X-ray examination at
Although many TAA cases are asymptomatic, symptoms such regular health check-ups in asymptomatic patients. Ascending
as hoarseness, difficulty swallowing, and vague back pain may aortic aneurysm usually presents a shadow protruding right-
occur. If TAA is detected, chest CT should first be per- ward continuing from the contour of the ascending aorta in the
formed to determine the most appropriate therapeutic strategy frontal view, whereas aortic arch aneurysm is often visualized
(Figure 7). as a tumorous shadow at the first left aortic arch in the frontal
Abdominal aortic aneurysm (AAA) may present symptoms view, and descending aortic aneurysm as a fusiform or round
such as a feeling of abdominal distension, constipation, non- shadow continuing from the contour of the aorta. In cases with
specific low back pain. A pulsatile mass in the abdomen is an AAA, calcification can sometimes be observed in the aneurys-
objective finding that leads to suspicion of AAA in some cases. mal wall, indicating the presence of an aneurysm.
Abdominal ultrasonography is the simplest and non-invasive
evaluation technique for initial diagnosis (Figure 8). The pa- (2) Aortic Dissection
tient should be followed up by ultrasonography or CT to de- In cases with acute aortic dissection, mediastinal shadow en-
Figure 6. Diagnosis and management algorithm for acute aortic dissection. ACS, acute coronary syndrome; CT, computed to-
mography; CRP, C-reactive protein; ECG, electrocardiograph; Hb, hemoglobin; ICU, intensive care unit; s/o, subjective symptoms/
objective signs; TEE, transesophageal echocardiography; WBC, white blood cell.
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796 JCS Joint Working Group
Figure 7. Diagnosis of thoracic aortic aneurysm. CT, computed tomography.
largement can be seen on plain chest X-rays, although this find- (3) C T of Ruptured Aortic Aneurysm and Impending
ing is nonspecific. Medial deviation of calcification of the in- Rupture
tima of the aortic wall is a finding suggestive of dissection.22 When aortic aneurysm rupture is suspected, CT is useful if the
patient’s condition allows time for such examination. It is im-
2. CT portant to carefully interpret CT images to avoid overlooking
(1) Method any minor bleeding.
For CT of aortic aneurysm or aortic dissection, plain CT im-
ages and early contrast-enhanced images are indispensable, (4) CT of Aortic Dissection
and late contrast-enhanced images should be added depending CT is a highly reliable, non-invasive examination for diagnos-
on the need in individual cases. Plain CT is useful for deter- ing dissection. This examination is indispensable for diagnos-
mining the degree of mural calcification, presence/absence of ing aortic dissection because it allows objective evaluation of
medial deviation, identifying hematoma in the false lumen in the whole aorta within a short period of time in response to an
cases with non-communicating dissection, and evaluating the urgent need.
high-density area in the mural thrombus suggesting impending On plain CT, deviation of intimal calcification is a critical
rupture of an aortic aneurysm.23 point in diagnosis.
(2) CT of Aortic Aneurysm 1) Communicating Dissection
CT allows us to identify an aneurysm and provides data on the In some cases with communicating dissection, blood flow in
size and extent of the aneurysm, calcification and the status of the false lumen is so slow that the false lumen cannot be visu-
the aneurysmal wall (e.g., inflammatory aortic aneurysm), the alized in the early-phase contrast, but inflow of the contrast
amount and status of the mural thrombus, the relationship agent is observed in the late-phase contrast. Therefore, it is
between the aneurysm and surrounding organs, and the posi- necessary to obtain late contrast-enhanced images as well. The
tional relationship between the aneurysm and major aortic entry is recognized as a rupture of the flap.
branches. Although the diameter of the aneurysm is an impor-
tant factor in determining whether surgery is indicated, “the 2) Non-Communicating Dissection
maximum minor-axis diameter” should be used in principle for CT images of non-communicating dissection are characterized
CT evaluation.24 by the presence of a false lumen filled with clotted blood or
hematoma in the acute phase. This false lumen is visualized as
a crescent-shaped or annular shadow resembling the mural
Circulation Journal Vol.77, March 2013
JCS Guidelines for Aortic Aneurysm and Aortic Dissection 797
Figure 8. Diagnosis of abdominal aortic aneurysm. CT, computed tomography.
thrombus that extends markedly in the direction of the long Body surface ultrasonography allows observation of the aortic
axis of the aorta. When detected early, this shadow may show root, ascending aorta, aortic arch, brachiocephalic artery, left
higher density than the true lumen on plain CT. Contrast-en- common carotid artery, and left subclavian artery. In addition,
hanced CT does not allow visualization of the inside of the arterial branches from the abdominal aorta, i.e., the celiac ar-
closed false lumen. tery, superior mesenteric artery, renal artery, and common iliac
artery, can also be observed. Transesophageal echocardiogra-
3) Ulcer-Like Projection-Type Dissection phy can clearly visualize the area from the aortic root to the
CT shows ULP as a localized luminal protrusion into the closed ascending aorta, aortic arch, and descending aorta.
false lumen.
1. Aortic Aneurysm
4) Diagnosis of Complications First, in imaging an aortic aneurysm, it is necessary to obtain
Complications of aortic dissection often include serious condi- the long axis and short axis views of the aorta on the body
tions such as rupture, cardiac tamponade, and ischemia in or- surface ultrasonography to observe the diameter of the aorta,
gans or limbs. On CT evaluation, it is important to pay atten- shape of the aneurysm, positional relationship with branching
tion to the presence/absence of fluid accumulation around the of blood vessels, and the nature of the lumen and wall. Be-
heart, the relationship between arterial branches and the dis- cause there is a possibility that the aorta is flexed or deviated,
secting lumen, and the presence/absence of extension of the the maximum minor-axis diameter should be measured when
dissection into arterial branches. the short axis view is used.
3. Angiography 2. Aortic Dissection
The role of angiography including digital subtraction angiog- Ultrasonography is very useful for rapid diagnosis of aortic
raphy (DSA) in the diagnosis of aortic aneurysm or aortic dis- dissection, and can be used when it is difficult to use contrast
section is diminishing. agents because of issues such as renal dysfunction or allergy
to contrast agents. In particular, body surface ultrasonography
allows evaluation of branch dissection and complications of
3. Ultrasonography
dissection, in addition to simple and non-invasive diagnosis of
dissection. It is very important to evaluate cardiac tamponade
Body surface ultrasonography and transesophageal echocar- (a complication of Stanford type A dissection), aortic valve
diography that are minimally invasive and provide large regurgitation, progression of dissection into a branch, blood
amounts of information are useful for visualizing the aorta. flow, and cardiac function.
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798 JCS Joint Working Group
cause it is very time-consuming and has limitations in patient
4. MRI
monitoring.
1. Imaging Procedures25–28 3. Safety of Implanted Devices and Metals
(1) MRI The safety of individual devices should be confirmed by refer-
Magnetic resonance imaging (MRI) allows evaluation of any ences, the website (www.mrisafety.com), and the latest pack-
section of the vascular wall and lumen without the use of con- age insert.
trast agents. On the other hand, this procedure requires a long
imaging time and may have artifacts due to turbulent flow or
5. Identification of Adamkiewicz Artery
delayed flow or respiration-related artifacts.
(2) Cine MRI To avoid paraplegia, one of the most serious complications
This imaging technique allows evaluation of hemodynamics of surgery involving the thoracic (abdominal) aorta, attempts
without the use of contrast agents. However, a long imaging have been made to identify the anatomical position of the
time is required, and information is basically limited to a sin- Adamkiewicz artery by diagnostic imaging prior to surgery.
gle section. Although CT and MRI have their own advantages and disad-
vantages, it has been reported that diagnostic capability reach-
(3) MRA es 90% for the Adamkiewicz artery if both CT and MRI can
Magnetic resonance angiography (MRA) techniques are broad- be performed in a particular patient.33 This data is approxi-
ly classified into the time-of-flight (TOF) method, phase-con- mately equivalent to that for angiography.34
trast (PC) method, fresh blood imaging method in which a
contrast agent is not used, and contrast-enhanced MRA in which 1. CT
a contrast agent is used. Among these techniques, the most To visualize the Adamkiewicz artery by CT, the inside of the
common procedure for the aorta is contrast-enhanced MRA, vertebral canal should be observed in an oblique coronal view
which can provide good views of the blood flow in the lumen by the multiplanar reformation (MPR) method using multi-
when evaluating the flexed part and the turbulent part. In com- detector-row CT (MDCT).33,35–37 Because the Adamkiewicz
parison with the TOF or PC method, contrast-enhanced MRA artery makes a distinguishable “hairpin turn” before conflu-
is advantageous in that a shorter imaging time is required, ence to the anterior spinal artery, it serves as a landmark for
there is high spatial resolution, and an arbitrary section can be identification of the artery. The continuity of the route from
set for imaging. the aorta to the intercostal (lumbar) artery, its posterior branch,
root-medullary vein, Adamkiewicz artery, and anterior spinal
2. Clinical Application1,26,27,29–32 artery should be observed as a traversable single blood vessel
MRI is more advantageous than CT in that it requires no X-ray route by the curved planar reformation (CPR) method.33,35,36
exposure, allows non-contrast imaging in patients with severe
renal dysfunction, and enables the lumen to be evaluated in 2. MRA
cases with severely calcified lesions. On the other hand, its dis- Images should be obtained by contrast-enhanced MRA.
advantages include lower spatial resolution, inability to visual- There are two MRA techniques available for visualizing the
ize osseous structures due to the lack of information on calci- Adamkiewicz artery.38 One is high spatial resolution MRA that
fication, and difficulty with responding to emergency cases focuses on spatial resolution,33,36 and the other is time-resolved
because of the long imaging time. MRA that focuses on time resolution.39,40 Time-resolved MRA
MRI is not recommended for diagnosis of acute aortic an- is more common at present.
eurysm in patients in a poor general medical condition, be-
III Choice of Treatment Methods
because the prognosis with medical treatment alone is ex-
1. Aortic Dissection (Recommendations for
tremely poor.
Choosing Treatment Methods for Acute
Aortic Dissection: Tables 3, and 4)
(2) Stanford Type B Acute Aortic Dissection
Because Stanford type B acute aortic dissection follows a bet-
In the treatment of aortic dissection, choosing between medi- ter natural course than type A acute aortic dissection, medical
cal and surgical treatment is the most important decision af- treatment is generally chosen as the initial management strat-
fecting prognosis. egy. On the other hand, surgical treatment is necessary for
patients with complications such as rupture, refractory pain, or
1. Treatment in the Acute Phase organ ischemia because the prognosis is extremely poor.44 How-
(1) Stanford Type A Acute Aortic Dissection ever, because in-hospital mortality after surgical treatment in
Stanford type A aortic dissection in which dissection extends the acute phase is not low,44 a good alternative to the currently
to the ascending aorta is a condition that has an extremely poor available surgical treatments is awaited. In recent years, endo-
prognosis. Mortality is reported to be 1 to 2% per hour after vascular treatment has been achieving favorable results as a
the onset of symptoms.41 The main causes of death include therapeutic option for acute type B aortic dissection,45–47 and is
rupture, cardiac tamponade, circulatory failure, cerebral infarc- now becoming a first-line therapy for patients with acute type
tion and intestinal tract ischemia.42,43 In general, surgical treat- B aortic dissection who have fatal complications.
ment, i.e., emergency surgery, is indicated for this condition,
Circulation Journal Vol.77, March 2013