Acc/aha pocket Guideline Based on the 2010 accf/aha/aats



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Thoracic Aorta

Range of Reported 

Mean (cm)

Reported SD 

(cm)

Assessment 

Method

Root (female)

3.50 to 3.72

0.38


CT

Root (male)

3.63 to 3.91

0.38


CT

Ascending (female, 

male)

2.86


NA

CXR


Mid-descending 

(female)


2.45 to 2.64

0.31


CT

Mid-descending 

(male)

2.39 to 2.98



0.31

CT

Diaphragmatic 



(female)

2.40 to 2.44

0.32

CT

Diaphragmatic 



(male)

2.43 to 2.69

0.27 to 0.40

CT, arteriography

CT indicates computed tomographic imaging; CXR, chest x-ray; and NA, not applicable.

Reprinted with permission from Johnston KW, Rutherford RB, Tilson MD, et al. Suggested standards for 

reporting on arterial aneurysms. Subcommittee on Reporting Standards for Arterial Aneurysms, Ad Hoc 

Committee on Reporting Standards, Society for Vascular Surgery and North American Chapter, 

International Society for Cardiovascular Surgery. J Vasc Surg. 1991;13:452–8. 


18

4. Recommendations for Genetic Syndromes 

Class I 

1. 

An echocardiogram is recommended at the time 

of diagnosis of Marfan syndrome to determine the 

aortic root and ascending aortic diameters and 6 

months thereafter to determine the rate of enlarge-

ment of the aorta. (LOE: C)



2. 

Annual imaging is recommended for patients with 

Marfan syndrome if stability of the aortic diameter is 

documented. If the maximal aortic diameter is 4.5 

cm or greater, or if the aortic diameter shows 

significant growth from baseline, more frequent 

imaging should be considered. (LOE: C)


19

3. 

Patients with Loeys-Dietz syndrome or a 

confirmed genetic mutation known to predispose to 

aortic aneurysms and aortic dissections (TGFBR1, 



TGFBR2, FBN1, ACTA2, or MYH11) should undergo 

complete aortic imaging at initial diagnosis and 6 

months thereafter to establish if enlargement is 

occurring. (LOE: C)



4. 

Patients with Loeys-Dietz syndrome should have 

yearly magnetic resonance imaging from the 

cerebrovascular circulation to the pelvis. (LOE: B) 



5. 

Patients with Turner syndrome should undergo 

imaging of the heart and aorta for evidence of 

bicuspid aortic valve, coarctation of the aorta, or 

dilatation of the ascending thoracic aorta. If initial 

imaging is normal and there are no risk factors for 

aortic dissection, repeat imaging should be performed 

every 5 to 10 years or if otherwise clinically indicated. 

If abnormalities exist, annual imaging or follow-up 

imaging should be done. (LOE: C)



20

Class IIa 



1. 

It is reasonable to consider surgical repair of the 

aorta in all adult patients with Loeys-Dietz syn-

drome or a confirmed TGFBR1 or TGFBR2 mutation 

and an aortic diameter of 4.2 cm or greater by trans-

esophageal echocardiogram (internal diameter) or 

4.4 to 4.6 cm or greater by computed tomographic 

imaging and/or magnetic resonance imaging (exter-

nal diameter). (LOE: C)

2. 

For women with Marfan syndrome contemplating 

pregnancy, it is reasonable to prophylactically 

replace the aortic root and ascending aorta if the 

diameter exceeds 4.0 cm. (LOE: C)

3. 

If the maximal cross-sectional area in square 

centimeters of the ascending aorta or root divided 

by the patient’s height in meters exceeds a ratio of 

10, surgical repair is reasonable because shorter 

patients have dissection at a smaller size and 15% of 

patients with Marfan syndrome have dissection at a 

size smaller than 5.0 cm. (LOE: C)

Class IIb 

1. 

In patients with Turner syndrome with additional 

risk factors, including bicuspid aortic valve, coarcta-

tion of the aorta, and/or hypertension, and in pa-

tients who attempt to become pregnant or who be-

come pregnant, it may be reasonable to perform im-

aging of the heart and aorta to help determine the 

risk of aortic dissection. (LOE: C)



21

Table 4. 

Gene Defects Associated With Familial Thoracic 

Aortic Aneurysm and Dissection



Defective Gene 

Leading to 

Familial Thoracic 

Aortic Aneurysms 

and Dissection

Contribution 

to Familial 

Thoracic Aortic 

Aneurysms 

and Dissection

Associated 

Clinical 

Features

Comments on 

Aortic Disease

TGFBR2 

mutations

4%

Thin, 


translucent 

skin


Arterial or 

aortic 


tortuosity 

Aneurysm of 

arteries

Multiple aortic 

dissections 

documented at 

aortic diameters 

<5.0 cm

MYH11 

mutations

1%

Patent ductus 



arteriosus

Patient with 

documented 

dissection at 4.5 

cm

ACTA2 mutations

14%


Livedo 

reticularis

Iris flocculi

Patent ductus 

arteriosus

Bicuspid aortic 

valve

Two of 13 



patients with 

documented 

dissections <5.0 

cm

ACTA2 indicates actin, alpha 2, smooth muscle aortaMYH11, smooth muscle specific beta-myosin 

heavy chain; and TGFBR2, transforming growth factor-beta receptor type II.


22

Table 5. 

Genetic Syndromes Associated With Thoracic Aortic Aneurysm         and Dissection

Genetic Syndrome

Common Clinical Features

Genetic Defect

Diagnostic Test

Comments on Aortic Disease

Marfan syndrome

Skeletal features (see text) 

Ectopia lentis

Dural ectasia

FBN1 mutations*

Ghent diagnostic criteria 

DNA for sequencing

Surgical repair when the aorta reaches 5.0 cm unless 

there is a family history of AoD at <5.0 cm, a rapidly 

expanding aneurysm or presence or significant aortic 

valve regurgitation

Loeys-Dietz syndrome

Bifid uvula or cleft palate 

Arterial tortuosity Hypertelorism

Skeletal features similar to MFS

Craniosynostosis

Aneurysms and dissections of 

other arteries



TGFBR2 or TGFBR1 

mutations

DNA for sequencing

Surgical repair recommended at an aortic diameter of 

≥4.2 cm by TEE (internal diameter) or 4.4 to ≥4.6 cm by 

CT and/or MR (external diameter)

Ehlers-Danlos syndrome, 

vascular form

Thin, translucent skin 

Gastrointestinal rupture

Rupture of the gravid uterus

Rupture of medium-sized to large 

arteries

COL3A1 mutations

DNA for sequencing

Dermal fibroblasts for 

analysis of type III collagen

Surgical repair is complicated by friable tissues 

Noninvasive imaging recommended

Turner syndrome

Short stature

Primary amenorrhea

Bicuspid aortic valve

Aortic coarctation

Webbed neck, low-set ears, low 

hairline, broad chest

45,X karyotype

Blood (cells) for karyotype 

analysis


AoD risk is increased in patients with bicuspid aortic 

valve, aortic coarctation, hypertension, or pregnancy

* The defective gene at a second locus for MFS is TGFBR2 but the clinical phenotype as MFS is debated. 

AoD indicates aortic dissection; COL3A1, type III collagen; CT, computed tomographic imaging; FBN1, fibrillin 1;  

MFS, Marfan syndrome; MR, magnetic resonance imaging; TEE, transesophageal echocardiogram; TGFBR1,  

transforming growth factor-beta receptor type I; and TGFBR2, transforming growth factor-beta receptor type II.



23

Table 5. 

Genetic Syndromes Associated With Thoracic Aortic Aneurysm         and Dissection

Genetic Syndrome

Common Clinical Features

Genetic Defect

Diagnostic Test

Comments on Aortic Disease

Marfan syndrome

Skeletal features (see text) 

Ectopia lentis

Dural ectasia

FBN1 mutations*

Ghent diagnostic criteria 

DNA for sequencing

Surgical repair when the aorta reaches 5.0 cm unless 

there is a family history of AoD at <5.0 cm, a rapidly 

expanding aneurysm or presence or significant aortic 

valve regurgitation

Loeys-Dietz syndrome

Bifid uvula or cleft palate 

Arterial tortuosity Hypertelorism

Skeletal features similar to MFS

Craniosynostosis

Aneurysms and dissections of 

other arteries



TGFBR2 or TGFBR1 

mutations

DNA for sequencing

Surgical repair recommended at an aortic diameter of 

≥4.2 cm by TEE (internal diameter) or 4.4 to ≥4.6 cm by 

CT and/or MR (external diameter)

Ehlers-Danlos syndrome, 

vascular form

Thin, translucent skin 

Gastrointestinal rupture

Rupture of the gravid uterus

Rupture of medium-sized to large 

arteries

COL3A1 mutations

DNA for sequencing

Dermal fibroblasts for 

analysis of type III collagen

Surgical repair is complicated by friable tissues 

Noninvasive imaging recommended

Turner syndrome

Short stature

Primary amenorrhea

Bicuspid aortic valve

Aortic coarctation

Webbed neck, low-set ears, low 

hairline, broad chest

45,X karyotype

Blood (cells) for karyotype 

analysis


AoD risk is increased in patients with bicuspid aortic 

valve, aortic coarctation, hypertension, or pregnancy

* The defective gene at a second locus for MFS is TGFBR2 but the clinical phenotype as MFS is debated. 

AoD indicates aortic dissection; COL3A1, type III collagen; CT, computed tomographic imaging; FBN1, fibrillin 1;  

MFS, Marfan syndrome; MR, magnetic resonance imaging; TEE, transesophageal echocardiogram; TGFBR1,  

transforming growth factor-beta receptor type I; and TGFBR2, transforming growth factor-beta receptor type II.



24

5. Recommendations for Familial Thoracic 

Aortic Aneurysms and Dissections 

Class I 


1. 

Aortic imaging is recommended for first-degree 

relatives of patients with thoracic aortic aneurysm 

and/or dissection to identify those with asymptom-

atic disease. (LOE: B)

2. 

If the mutant gene (FBN1, TGFBR1, TGFBR2, 



COL3A1, ACTA2, MYH11) associated with aortic 

aneurysm and/or dissection is identified in a patient, 

first-degree relatives should undergo counseling and 

testing. Then, only the relatives with the genetic 

mutation should undergo aortic imaging. (LOE: C)

  

Class IIa 



1. 

If one or more first-degree relatives of a patient 

with known thoracic aortic aneurysm and/or dissec-

tion are found to have thoracic aortic dilatation, an-

eurysm, or dissection, then imaging of second-de-

gree relatives is reasonable. (LOE: B) 



25

2. 

Sequencing of the ACTA2 gene is reasonable in 

patients with a family history of thoracic aortic 

aneurysms and/or dissections to determine if ACTA2 

mutations are responsible for the inherited 

predisposition. (LOE: B)

Class IIb 

1. 

Sequencing of other genes known to cause famil-

ial thoracic aortic aneurysms and/or dissection 

(TGFBR1, TGFBR2, MYH11) may be considered in pa-

tients with a family history and clinical features as-

sociated with mutations in these genes. (LOE: B)



2. 

If one or more first-degree relatives of a patient 

with known thoracic aortic aneurysm and/or 

dissection are found to have thoracic aortic 

dilatation, aneurysm, or dissection, then referral to a 

geneticist may be considered. (LOE: C)



26

6. Recommendations for Bicuspid Aortic 

Valve and Associated Congenital Variants in 

Adults


Class I 

1. 

First-degree relatives of patients with a bicuspid 

aortic valve, premature onset of thoracic aortic dis-

ease with minimal risk factors, and/or a familial 

form of thoracic aortic aneurysm and dissection 

should be evaluated for the presence of a bicuspid 

aortic valve and asymptomatic thoracic aortic dis-

ease. (LOE: C) 



2. 

All patients with a bicuspid aortic valve should 

have both the aortic root and ascending thoracic 

aorta evaluated for evidence of aortic dilatation. 



(LOE: B)

27

7. Recommendations for Estimation of 

Pretest Risk of Thoracic Aortic Dissection

Class I 


1. 

Providers should routinely evaluate any patient 

presenting with complaints that may represent acute 

thoracic aortic dissection to establish a pretest risk 

of disease that can then be used to guide diagnostic 

decisions. This process should include specific ques-

tions about medical history, family history, and pain 

features as well as a focused examination to identify 

findings that are associated with aortic dissection, 

including: 

a.  High-risk conditions and historical features 

(LOE: B):

Marfan syndrome, Loeys-Dietz syndrome, 



vascular Ehlers-Danlos syndrome, Turner 

syndrome, or other connective tissue disease. 

Patients with mutations in genes known to 



predispose to thoracic aortic aneurysms and 

dissection, such as FBN1, TGFBR1, TGFBR2, 



ACTA2, and MYH11

Family history of aortic dissection or thoracic 



aortic aneurysm. 

Known aortic valve disease. 



Recent aortic manipulation (surgical or 

catheter-based). 

Known thoracic aortic aneurysm. 



28

b.  High-risk chest, back or abdominal pain features 



(LOE: B):

•  Pain that is abrupt or instantaneous in onset. 

•  Pain that is severe in intensity. 

•  Pain that has a ripping, tearing, stabbing, or 

sharp quality. 

c.  High-risk examination features (LOE: B):

•  Pulse deficit. 

•  Systolic blood pressure limb differential greater 

than 20 mm Hg. 

•  Focal neurologic deficit. 

•  Murmur of aortic regurgitation (new).

2. 

Patients presenting with sudden onset of severe 

chest, back and/or abdominal pain, particularly 

those less than 40 years of age, should be 

questioned about a history and examined for 

physical features of Marfan syndrome, Loeys-Dietz 

syndrome, vascular Ehlers-Danlos syndrome, Turner 

syndrome, or other connective tissue disorder 

associated with thoracic aortic disease. (LOE: B)

3. 

Patients presenting with sudden onset of severe 

chest, back, and/or abdominal pain should be 

questioned about a history of aortic pathology in 

immediate family members as there is a strong 

familial component to acute thoracic aortic disease.  



(LOE: B) 

29

4. 

Patients presenting with sudden onset of severe 

chest, back and/or abdominal pain should be 

questioned about recent aortic manipulation 

(surgical or catheter-based) or a known history of 

aortic valvular disease, as these factors predispose 

to acute aortic dissection. (LOE: C) 

5. 

In patients with suspected or confirmed aortic 

dissection who have experienced a syncopal 

episode, a focused examination should be performed 

to identify associated neurologic injury or the 

presence of pericardial tamponade. (LOE: C) 



6. 

All patients presenting with acute neurologic 

complaints should be questioned about the presence 

of chest, back, and/or abdominal pain and checked 

for peripheral pulse deficits as patients with 

dissection-related neurologic pathology are less 

likely to report thoracic pain than the typical aortic 

dissection patient. (LOE: C)



30

Figur


e 2.

 

AoD Evaluation P



athway

.

ACS indicates acute coronary syndrome;



 AoD

, aortic dissection;

 BP

, blood pressure;



 CNS

, central nervous system;

 CT

, computed tomographic imaging;



 CXR,

 chest 


x-ray;

 EKG


, electrocardiogram;

 MR,


 magnetic resonance imaging;

 STEMI,


 ST

-elev


ated myocardial infarction;

 TAD;


 thoracic aortic disease;

 and 


TEE,

 

transesophageal echocardiogram.



 

31

Figur


e 2.

 

AoD Evaluation P



athway

.

ACS indicates acute coronary syndrome;



 AoD

, aortic dissection;

 BP

, blood pressure;



 CNS

, central nervous system;

 CT

, computed tomographic imaging;



 CXR,

 chest 


x-ray;

 EKG


, electrocardiogram;

 MR,


 magnetic resonance imaging;

 STEMI,


 ST

-elev


ated myocardial infarction;

 TAD;


 thoracic aortic disease;

 and 


TEE,

 

transesophageal echocardiogram.



 

32

Table 6. 

Risk Factors for Development of Thoracic Aortic 

Dissection



Conditions Associated With Increased Aortic Wall Stress

Hypertension, particularly if uncontrolled

Pheochromocytoma

Cocaine or other stimulant use

Weight lifting or other Valsalva maneuver

Trauma


Deceleration or torsional injury (eg, motor vehicle crash, fall)

Coarctation of the aorta



Conditions Associated With Aortic Media Abnormalities

Genetic


Marfan syndrome

Ehlers-Danlos syndrome, vascular form

Bicuspid aortic valve (including prior aortic valve replacement)

Turner syndrome

Loeys-Dietz syndrome

Familial thoracic aortic aneurysm and dissection syndrome

Inflammatory vasculitides

Takayasu arteritis

Giant cell arteritis

Behçet arteritis

Other

Pregnancy



Polycystic kidney disease

Chronic corticosteroid or immunosuppression agent administration

Infections involving the aortic wall either from bacteremia or extension 

of adjacent infection



33

Figure 3. 

Aortic Dissection Classification: DeBakey and 

Stanford Classifications.

Reprinted with permission from the Cleveland Clinic Foundation.


34

8. Initial Evaluation and Management of 

Acute Thoracic Aortic Disease

8.1. Recommendations for Screening Tests

Class I 


1. 

An electrocardiogram should be obtained on all 

patients who present with symptoms that may rep-

resent acute thoracic aortic dissection. 

a.  Given the relative infrequency of dissection-

related coronary artery occlusion, the presence of 

ST-segment elevation suggestive of myocardial 

infarction should be treated as a primary cardiac 

event without delay for definitive aortic imaging 

unless the patient is at high risk for aortic 

dissection. (LOE: B)

2. 

The role of chest x-ray in the evaluation of 

possible thoracic aortic disease should be directed 

by the patient’s pretest risk of disease as follows. 

a.  Intermediate risk: Chest x-ray should be 

performed on all intermediate-risk patients, as it 

may establish a clear alternate diagnosis that will 

obviate the need for definitive aortic imaging. 



(LOE: C)

b.  Low risk: Chest x-ray should be performed on all 

low-risk patients, as it may either establish an 

alternative diagnosis or demonstrate findings 

that are suggestive of thoracic aortic disease, 

indicating the need for urgent definitive aortic 

imaging. (LOE: C)


35

3. 

Urgent and definitive imaging of the aorta using 

transesophageal echocardiogram, computed 

tomographic imaging, or magnetic resonance 

imaging is recommended to identify or exclude 

thoracic aortic dissection in patients at high risk for 

the disease by initial screening. (LOE: B)

Class III 



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