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Guideline Summary
Guideline Title
2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.
Bibliographic Source(s)
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM, American College of Cardiology Foundation/American Heart Association Task Force [trunc], American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. A report of the American College of Cardiology Foundation/American Heart Association Task Force [trunc]. J Am Coll Cardiol. 2010 Apr 6;55(14):e27-e129. [827 references] PubMed External Web Site Policy
Guideline Status

This is the current release of the guideline.

Scope

Disease/Condition(s)
  • Thoracic aortic disease, encompassing a broad range of degenerative, structural, acquired, genetic-based, and traumatic disease states and presentation such as Takayasu arteritis, giant cell arteritis, Behçet disease, ankylosing spondylitis (spondyloarthropathies), and infective thoracic aortic aneurysms
  • Diseases involving any or all parts of the thoracic aorta, with the exception of aortic valve diseases and including the abdominal aorta when contiguous thoracic aortic diseases are present
Guideline Category
Counseling
Diagnosis
Evaluation
Management
Prevention
Risk Assessment
Screening
Treatment
Clinical Specialty
Cardiology
Critical Care
Emergency Medicine
Family Practice
Internal Medicine
Medical Genetics
Obstetrics and Gynecology
Pediatrics
Preventive Medicine
Radiology
Surgery
Thoracic Surgery
Intended Users
Advanced Practice Nurses
Hospitals
Physician Assistants
Physicians
Guideline Objective(s)
  • To assist healthcare providers in clinical decision making by describing a range of generally acceptable approaches for diagnosis, management, and prevention of specific diseases or conditions
  • To define practices that meet the needs of most patients in most circumstances
  • To provide the practitioner with a sufficient description of background information, diagnostic modalities, and treatment strategies so that appropriate care of patients can be facilitated and better understood
  • To improve the health outcomes and quality of life for all patients with thoracic aortic disease
Target Population

Patients with known or suspected thoracic aortic disease

Interventions and Practices Considered

Diagnosis/Screening

  1. Computed tomographic imaging
  2. Magnetic resonance imaging
  3. Transesophageal echocardiography
  4. Medical history
  5. Family history
  6. Pain features
  7. Focused examination
  8. Gene sequencing and evaluation of patient relatives
  9. Electrocardiogram
  10. Chest X-ray
  11. Motor or somatosensory evoked potentials
  12. Preoperative evaluation (pulmonary function testing, cardiac catheterization, 24-hour Holter monitoring)

Management/Treatment/Prevention

  1. Pharmacologic management
    • Intravenous beta blockade
    • Nondihydropyridine calcium channel-blocking agents
    • Angiotensin-converting enzyme inhibitors and/or other vasodilators
    • High-dose corticosteroids with or without additional inflammatory agents
  2. Elective revascularization
  3. Atherosclerosis risk reduction
    • Control of blood pressure (angiotensin-converting enzyme inhibitors, angiotensin receptor blockers)
    • Lipid profile optimization (statins)
    • Smoking cessation
  4. Surgical repair
    • Aortic root replacement
    • Aortic replacement
    • Aortic valve resuspension
    • Valve sparing root replacement
    • Concomitant coronary artery bypass graft (CABG)
    • Endovascular stent grafting
  5. Excision of the sinuses in combination with a modified David reimplantation
  6. Stroke prevention: statins, oral anticoagulation therapy
  7. Management of chronic aortic diseases in pregnancy
  8. Choice of anesthetic and patient monitoring technique
  9. Transfusion management and anticoagulation
Major Outcomes Considered
  • Sensitivity, specificity, accuracy, and predictive value of diagnostic and screening tests
  • Risk of complications from diagnostic and screening tests
  • Risk and incidence of thoracic aortic disease, including aneurysm, aortic dissection, intramural hematoma, penetrating atherosclerotic ulcer, pseudoaneurysms of the thoracic aorta, and traumatic rupture of the thoracic aorta
  • Symptomatology of thoracic aortic disease, including pain
  • Disease-related complications, including cardiac, syncope, neurologic, pulmonary, gastrointestinal, blood pressure, and heart rate complications
  • Disease-related morbidity and mortality
  • Response to treatment for thoracic aortic disease and associated diseases and conditions
  • Safety of treatment
  • Rate of disease progression
  • Treatment-related complications
  • Treatment-related morbidity and mortality
  • Survival
  • Length of intensive care stay
  • Length of hospital stay

Methodology

Methods Used to Collect/Select the Evidence
Hand-searches of Published Literature (Primary Sources)
Hand-searches of Published Literature (Secondary Sources)
Searches of Electronic Databases
Description of Methods Used to Collect/Select the Evidence

The writing committee conducted a comprehensive search of the medical and scientific literature through the use of PubMed/MEDLINE through 2009. Searches were limited to publications written in the English language. Compiled reports were reviewed and additional articles were provided by committee members. Specifically targeted searches were conducted on the following subtopics: acute aortic dissection, ankylosing spondylitis, aortic dissection and litigation, aortic neoplasm, aortic tumors, Behçet disease, bicuspid aortic valve, calcified aorta, chronic dissection, coarctation of the aorta, D-dimer, dissecting aneurysm, Ehlers-Danlos syndrome, endovascular and aortic aneurysms, medial degeneration, porcelain aorta, giant cell arteritis, imaging and thoracic aortic disease, inflammatory disease, intramural hematoma, Loeys-Dietz syndrome, Marfan syndrome, Noonan syndrome, penetrating aortic ulcer, polycystic kidney disease, thoracic and aortic aneurysms, thoracic aortic disease and patient care, thoracic aortic disease and surgery, thoracic aorta and Kawasaki disease, Takayasu arteritis, thoracoabdominal and aorta or aortic disease, and Turner syndrome. In general, published manuscripts appearing in journals listed in Index Medicus were used as the evidence base. Published abstracts were used only for emerging information but were not used in the formulation of recommendations.

Number of Source Documents

More than 850 references were reviewed, with 829 used as the primary evidence base for the final guideline.

Methods Used to Assess the Quality and Strength of the Evidence
Weighting According to a Rating Scheme (Scheme Given)
Rating Scheme for the Strength of the Evidence

Applying Classification of Recommendations and Level of Evidence

  Size of Treatment Effect
  CLASS I

Benefit >>> Risk

Procedure/Treatment
SHOULD be performed/ administered
CLASS IIa

Benefit >> Risk
Additional studies with focused objectives needed


IT IS REASONABLE to perform procedure/administer treatment
CLASS IIb

Benefit ≥ Risk
Additional studies with broad objectives needed; additional registry data would be helpful


Procedure/Treatment
MAY BE CONSIDERED
CLASS III No Benefit
or Class III Harm
  Procedure/Test Treatment
COR III:
No Benefit
Not helpful No proven benefit
COR III:
Harm
Excess Cost without Benefit or Harmful Harmful to Patients
Estimate of Certainty (Precision) of Treatment Effect LEVEL A

Multiple populations evaluated*

Data derived from multiple randomized clinical trials or meta-analyses
  • Recommendation that procedure or treatment is useful/effective
  • Sufficient evidence from multiple randomized trials or meta-analyses
  • Recommendation in favor of treatment or procedure being useful/effective
  • Some conflicting evidence from multiple randomized trials or meta-analyses
  • Recommendation's usefulness/efficacy less well established
  • Greater conflicting evidence from multiple randomized trials or meta-analyses
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Sufficient evidence from multiple randomized trials or meta-analyses
LEVEL B

Limited populations evaluated*

Data derived from a single randomized clinical trials or nonrandomized studies
  • Recommendation that procedure or treatment is useful/effective
  • Evidence from single randomized trial or nonrandomized studies
  • Recommendation in favor of treatment or procedure being useful/effective
  • Some conflicting evidence from single randomized trial or nonrandomized studies
  • Recommendation's usefulness/efficacy less well established
  • Greater conflicting evidence from single randomized trial or nonrandomized studies
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Evidence from single randomized trial or nonrandomized studies
LEVEL C

Very limited populations evaluated*

Only consensus opinion of experts, case studies or standard of care
  • Recommendation that procedure or treatment is useful/effective
  • Only expert opinion, case studies, or standard of care
  • Recommendation in favor of treatment or procedure being useful/effective
  • Only diverging expert opinion, case studies, or standard of care
  • Recommendation's usefulness/efficacy less well established
  • Only diverging expert opinion, case studies, or standard of care
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Only expert opinion, case studies, or standard of care

*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.

Methods Used to Analyze the Evidence
Review of Published Meta-Analyses
Systematic Review with Evidence Tables
Description of the Methods Used to Analyze the Evidence

The committee reviewed and ranked evidence supporting current recommendations. The schema for classification of recommendations and level of evidence is summarized in the "Rating Scheme for the Strength of Evidence" field, which also illustrates how the grading system provides an estimate of the size of the treatment effect and an estimate of the certainty of the treatment effect.

To provide clinicians with a comprehensive set of data, whenever possible, the exact event rates in various treatment arms of clinical trials are presented to permit calculation of the absolute risk difference (ARD), number needed to harm (NNH); the relative treatment effects are described either as odds ratio (OR), relative risk (RR), or hazard ratio (HR) depending on the format in the original publication. Along with all other point statistics, confidence intervals (CIs) for those statistics are added when available.

The writing committee recognized that the evidence base for this guideline is less robust in terms of randomized clinical trials than prior American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines, particularly those focused on coronary artery disease and heart failure. Much of the evidence base for this topic consists of cohort studies and retrospective reviews, which largely emanate from centers with a specialized interest in specific types of thoracic aortic disease. The writing committee attempted to focus on providing the practitioner with recommendations for evaluation and treatment wherever possible and where controversy exists, identified as such in the text of the original guideline document.

Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations

The American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Task Force on Practice Guidelines methodology processes were followed to write the text and recommendations (see the "Availability of Companion Documents" field).

Experts in the subject under consideration have been selected from both organizations to examine subject-specific data and write guidelines in partnership with representatives from other medical practitioner and specialty groups. Writing committees are specifically charged to perform a formal literature review, weigh the strength of evidence for or against particular treatments or procedures, and include estimates of expected health outcomes where data exist. Patient-specific modifiers, comorbidities, and issues of patient preference that may influence the choice of tests or therapies are considered. When available, information from studies on cost is considered, but data on efficacy and clinical outcomes constitute the primary basis for recommendations in these guidelines.

Rating Scheme for the Strength of the Recommendations

See the "Rating Scheme for the Strength of the Evidence" field, above.

Cost Analysis

There are no firm data to indicate that overall costs of medical care are lower with endovascular procedures versus open surgical approach for descending thoracic aorta and thoracoabdominal aortic aneurysms. Although the costs of the initial hospitalization may be lower because of reduced operative time and a shorter length of stay, these benefits may be negated by the cost of the devices, the need for subsequent interventions, and the cost and dissatisfaction of repeated imaging studies, which are necessary in the postoperative period.

Method of Guideline Validation
External Peer Review
Internal Peer Review
Description of Method of Guideline Validation

This document was reviewed by 3 outside reviewers nominated by the American College of Cardiology Foundation (ACCF) and 2 outside reviewers nominated by the American Heart Association (AHA), as well as 1 or 2 reviewers from each of the following organizations: the American Association for Thoracic Surgery (AATS), American College of Physicians (ACP), American College of Emergency Physicians (ACEP), American College of Radiology (ACR), American Stroke Association (ASA), Society of Cardiovascular Anesthesiologists (SCA), Society for Cardiovascular Angiography and Interventions (SCAI), Society of Interventional Radiology (SIR), Society of Thoracic Surgeons (STS), and the Society for Vascular Medicine (SVM). It was also reviewed by 6 individual content reviewers—2 content reviewers from the ACCF Catheterization Committee and 1 content reviewer from the ACCF Interventional Council.

This document was approved for publication by the governing bodies of the ACCF and the AHA and the AATS, ACR, ASA, SCA, SCAI, SIR, STS, and the SVM and was endorsed by the North American Society for Cardiovascular Imaging.

Recommendations

Major Recommendations

Message from the Guideline Developers: The American College of Cardiology, American Heart Association, and the European Society of Cardiology are all in the process of completing updated versions of our Guidelines for Perioperative Care. Our respective writing committees are undertaking a careful analysis of all relevant validated studies and always incorporate appropriate new trials and meta-analyses into our evidence review. In the interim, our current joint position is that the initiation of beta blockers in patients who will undergo non-cardiac surgery should not be considered routine, but should be considered carefully by each patient's treating physician on a case-by-case basis. Please see the expression of concern which is free to view in Eur Heart J (2013) 34 (44): 3460; doi: 10.1093/eurheartj/eht431.

Definitions for the weight of the evidence (A-C) and classes of recommendations (I-III) are provided at the end of the "Major Recommendations" field.

Imaging Modalities

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 (see the table below). (Level of Evidence: C)

Table. Essential Elements of Aortic Imaging Reports

  1. The location at which the aorta is abnormal (see Section 2 in the original guideline document).
  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 intramural hematoma (IMH), penetrating atherosclerotic ulcer (PAU), and calcification.
  6. Extension of aortic abnormality into branch vessels, including dissection and aneurysm, and secondary evidence of end-organ injury (e.g., 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.
  1. 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 (Level of Evidence: C).
  2. 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. (Level of Evidence: C)
  3. Abnormalities of aortic morphology should be recognized and reported separately even when aortic diameters are within normal limits. (Level of Evidence: C)
  4. The finding of aortic dissection, aneurysm, traumatic injury and/or aortic rupture should be immediately communicated to the referring physician. (Level of Evidence: C)
  5. Techniques to minimize episodic and cumulative radiation exposure should be utilized whenever possible (Amis et al., 2007; Brenner & Hall, 2007). (Level of Evidence: 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. (Level of Evidence: C)

Genetic Syndromes Associated with Thoracic Aortic Aneurysms and Dissection

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 enlargement of the aorta. (Level of Evidence: 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. (Level of Evidence: C)
  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 (Pearson et al., 2008; Svensson et al., "Impact," 1989; Svensson et al., 2007; Zhu et al., 2006). (Level of Evidence: C)
  4. Patients with Loeys-Dietz syndrome should have yearly magnetic resonance imaging from the cerebrovascular circulation to the pelvis (LeMaire et al., 2007; Loeys et al., 2006; Williams et al., 2007). (Level of Evidence: 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 (Bondy & Turner Syndrome Study Group, 2007). 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. (Level of Evidence: C)

Class IIa

  1. It is reasonable to consider surgical repair of the aorta in all adult patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation and an aortic diameter of 4.2 cm or greater by transesophageal echocardiogram (internal diameter) or 4.4 to 4.6 cm or greater by computed tomographic imaging and/or magnetic resonance imaging (external diameter) (Loeys et al., 2006). (Level of Evidence: 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 (Pearson et al., 2008). (Level of Evidence: 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 less than 5.0 cm (Svensson & Khitin, 2002; Svensson et al., 2007; Gott et al., 1999). (Level of Evidence: C)

Class IIb

  1. In patients with Turner syndrome with additional risk factors, including bicuspid aortic valve, coarctation of the aorta, and/or hypertension, and in patients who attempt to become pregnant or who become pregnant, it may be reasonable to perform imaging of the heart and aorta to help determine the risk of aortic dissection. (Level of Evidence: C)

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 asymptomatic disease (Albornoz et al., 2006; Coady et al., 1999). (Level of Evidence: 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. (Level of Evidence: C)

Class IIa

  1. 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 imaging of second-degree relatives is reasonable (Albornoz et al., 2006). (Level of Evidence: B)
  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 (Pannu et al., 2005; Guo et al., 2007; Zhu et al., 2006; Loeys et al., 2006; Stheneur et al., 2008; Guo et al., 2009). (Level of Evidence: B)

Class IIb

  1. Sequencing of other genes known to cause familial thoracic aortic aneurysms and/or dissection (TGFBR1, TGFBR2, MYH11) may be considered in patients with a family history and clinical features associated with mutations in these genes (Pannu et al., 2005; Guo et al., 2007; Zhu et al., 2006; Loeys et al., 2006; Stheneur et al., 2008; Guo et al., 2009). (Level of Evidence: 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. (Level of Evidence: C)

Other Cardiovascular Conditions Associated with Thoracic Aortic Aneurysm and Dissection

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 disease 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 disease. (Level of Evidence: 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 (Braverman et al., 2005; Borger & David, 2005; Svensson, Blackstone, & Cosgrove, 2003; Svensson, 2008). (Level of Evidence: B)

Inflammatory Diseases Associated with Thoracic Aortic Disease

Recommendations for Takayasu Arteritis and Giant Cell Arteritis

Table. Inflammatory Diseases Associated with Thoracic Aortic Aneurysm and Dissection

Names Criteria Used in Diagnosis/Source When Is Diagnosis Established?

Takayasu arteritis (Arend et al., 1990)

Age of onset <40 y

Intermittent claudication

Diminished brachial artery pulse

Subclavian artery or aortic bruit

Systolic blood pressure (BP) variation of >10 mm Hg between arms

Aortographic evidence of aorta or aortic branch stenosis

≥3 criteria are present (sensitivity 90.5%; specificity 97.8%)

Giant cell arteritis (Hunder et al., 1990)

Age >50 y

Recent-onset localized headache

Temporary artery tenderness or pulse attenuation

Elevated erythrocyte sedimentation >50 mm/h

Arterial biopsy shows necrotizing vasculitis

≥3 criteria are present (sensitivity greater than 90%; specificity >90%)

Behçet disease ("Evaluation of diagnostic ['classification'] criteria," 1992)

Oral ulceration

Recurrent genital ulceration

Uveitis or retinal vasculitis

Skin lesions—erythema nodosum, pseudo-folliculitis, or pathergy

Oral ulceration plus 2 of the other 3 criteria

Ankylosing spondylitis (Goie et al., 1985)

Onset of pain <40 y

Back pain for >3 mo

Morning stiffness

Subtle symptom onset

Improvement with exercise

4 of the diagnostic criteria are present

Class I

  1. Initial therapy for active Takayasu arteritis and active giant cell arteritis should be corticosteroids at a high dose (prednisone 40 to 60 mg daily at initiation or its equivalent) to reduce the active inflammatory state (Kerr et al., 1994; Mazlumzadeh et al., 2006). (Level of Evidence: B)
  2. The success of treatment of patients with Takayasu arteritis and giant cell arteritis should be periodically evaluated to determine disease activity by repeated physical examination and either an erythrocyte sedimentation rate or C-reactive protein level (Ishikawa & Maetani, 1994; Kyle, Cawston, & Hazleman, 1989). (Level of Evidence: B)
  3. Elective revascularization of patients with Takayasu arteritis and giant cell arteritis should be delayed until the acute inflammatory state is treated and quiescent (Fields, et al., 2006). (Level of Evidence: B)
  4. The initial evaluation of Takayasu arteritis or giant cell arteritis should include thoracic aorta and branch vessel computed tomographic imaging or magnetic resonance imaging to investigate the possibility of aneurysm or occlusive disease in these vessels. (Level of Evidence: C)

Class IIa

  1. It is reasonable to treat patients with Takayasu arteritis receiving corticosteroids with an additional anti-inflammatory agent if there is evidence of progression of vascular disease, recurrence of constitutional symptoms, or re-elevation of inflammatory marker (Kerr et al., 1994). (Level of Evidence: C)

Acute Aortic Syndromes

Evaluation and Management of Acute Thoracic Aortic Disease

Initial Evaluation and Management

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 questions about medical history, family history, and pain features as well as a focused examination to identify findings that are associated with aortic dissection, including:
    1. High-risk conditions and historical features (Hagan et al., 2000; Coady et al., 1999; Januzzi et al., 2004; von Kodolitsch, Schwartz, & Nienaber, 2000) (Level of Evidence: 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
    1. High-risk chest, back, or abdominal pain features (Klompas, 2002; Hagan et al., 2000; Meszaros et al., 2000; Januzzi et al., 2004; von Kodolitsch, Schwartz, & Nienaber, 2000; Spittell et al., 1993; Mehta et al., "Acute type", 2002) (Level of Evidence: 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
    1. High-risk examination features (Klompas, 2002; Hagan et al., 2000; Gaul et al., 2007; Armstrong et al., 1998; von Kodolitsch, Schwartz, & Nienaber, 2000; Roberts et al., 2006) (Level of Evidence: B):
      • Pulse deficit
      • Systolic blood pressure limb differential greater than 20 mm Hg
      • Focal neurologic deficit
      • Murmur of aortic regurgitation (new)
  1. 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 (Januzzi et al., 2004). (Level of Evidence: B)
  2. 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 (Januzzi et al., 2004). (Level of Evidence: B)
  3. 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. (Level of Evidence: C)
  4. 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 (see Section 8.1.6 in the original guideline document). (Level of Evidence: C)
  5. 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 (Gaul et al., 2007) (see Section 8.1 in the original guideline document). (Level of Evidence: C)

Recommendations for Screening Tests

Class I

  1. An electrocardiogram should be obtained on all patients who present with symptoms that may represent acute thoracic aortic dissection.
    1. 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 (Klompas, 2002; Hagan et al., 2000; Antman et al., 2004). (Level of Evidence: 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:
    1. 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. (Level of Evidence: C)
    2. 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. (Level of Evidence: C)
  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 (Erbel et al., 1989; Yoshida et al., 2003; Sommer et al., 1996; Zeman et al., 1995; Shiga et al., 2006; Nienaber et al., 1993; Borner et al., 1984). (Level of Evidence: B)

Class III

  1. A negative chest x-ray should not delay definitive aortic imaging in patients determined to be high risk for aortic dissection by initial screening. (Level of Evidence: C)

Recommendations for Diagnostic Imaging Studies

Class I

  1. Selection of a specific imaging modality to identify or exclude aortic dissection should be based on patient variables and institutional capabilities, including immediate availability. (Level of Evidence: C)
  2. If a high clinical suspicion exists for acute aortic dissection but initial aortic imaging is negative, a second imaging study should be obtained (Svensson et al., 1999). (Level of Evidence: C)

Recommendations for Initial Management

Class I

  1. Initial management of thoracic aortic dissection should be directed at decreasing aortic wall stress by controlling heart rate and blood pressure as follows:
    1. In the absence of contraindications, intravenous beta blockade should be initiated and titrated to a target heart rate of 60 beats per minute or less. (Level of Evidence: C)
    2. In patients with clear contraindications to beta blockade, nondihydropyridine calcium channel-blocking agents should be used as an alternative for rate control. (Level of Evidence: C)
    3. If systolic blood pressures remain greater than 120 mm Hg after adequate heart rate control has been obtained, then angiotensin-converting enzyme inhibitors and/or other vasodilators should be administered intravenously to further reduce blood pressure that maintains adequate end-organ perfusion. (Level of Evidence: C)
    4. Beta blockers should be used cautiously in the setting of acute aortic regurgitation because they will block the compensatory tachycardia (American College of Cardiology/American Heart Association Task Force on Practice Guidelines et al., 2006). (Level of Evidence: C)

Class III

  1. Vasodilator therapy should not be initiated prior to rate control so as to avoid associated reflex tachycardia that may increase aortic wall stress, leading to propagation or expansion of a thoracic aortic dissection. (Level of Evidence: C)

Recommendations for Definitive Management

Class I

  1. Urgent surgical consultation should be obtained for all patients diagnosed with thoracic aortic dissection regardless of the anatomic location (ascending versus descending) as soon as the diagnosis is made or highly suspected. (Level of Evidence: C)
  2. Acute thoracic aortic dissection involving the ascending aorta should be urgently evaluated for emergent surgical repair because of the high risk of associated life-threatening complications such as rupture (Hagan et al., 2000). (Level of Evidence: B)
  3. Acute thoracic aortic dissection involving the descending aorta should be managed medically unless life-threatening complications develop (i.e., malperfusion syndrome, progression of dissection, enlarging aneurysm, inability to control blood pressure or symptoms) (Suzuki et al., 2003; Mehta et al., "Acute type", 2002; Estrera et al., 2006; Umana et al., 2002; Mehta et al., "Predicting death", 2002; Chiappini et al., 2005). (Level of Evidence: B)

Recommendation for Surgical Intervention for Acute Thoracic Aortic Dissection

Class I

  1. For patients with ascending thoracic aortic dissection, all of the aneurysmal aorta and the proximal extent of the dissection should be resected. A partially dissected aortic root may be repaired with aortic valve resuspension. Extensive dissection of the aortic root should be treated with aortic root replacement with a composite graft or with a valve sparing root replacement. If a DeBakey Type II dissection is present, the entire dissected aorta should be replaced. (Level of Evidence: C)

Principles of Treatment for Intramural Hematoma and Penetrating Atherosclerotic Ulcer

Recommendation for Intramural Hematoma without Intimal Defect

Class IIa

  1. It is reasonable to treat intramural hematoma similar to aortic dissection in the corresponding segment of the aorta. (Level of Evidence: C)

Thoracic Aortic Aneurysms

General Approach to the Patient

Recommendation for History and Physical Examination for Thoracic Aortic Disease

Class I

  1. For patients presenting with a history of acute cardiac and noncardiac symptoms associated with a significant likelihood of thoracic aortic disease, the clinician should perform a focused physical examination, including a careful and complete search for arterial perfusion differentials in both upper and lower extremities, evidence of visceral ischemia, focal neurologic deficits, a murmur of aortic regurgitation, bruits, and findings compatible with possible cardiac tamponade (Isselbacher, 2008; Libby et al, 2007; Townsend et al., 2008). (Level of Evidence: C)

General Medical Treatment and Risk Factor Management for Patients with Thoracic Aortic Disease

Recommendation for Medical Treatment of Patients with Thoracic Aortic Diseases

Class I

  1. Stringent control of hypertension, lipid profile optimization, smoking cessation, and other atherosclerosis risk-reduction measures should be instituted for patients with small aneurysms not requiring surgery, as well as for patients who are not considered to be surgical or stent graft candidates. (Level of Evidence: C)

Recommendations for Blood Pressure Control

Class I

  1. Antihypertensive therapy should be administered to hypertensive patients with thoracic aortic diseases to achieve a goal of less than 140/90 mm Hg (patients without diabetes) or less than 130/80 mm Hg (patients with diabetes or chronic renal disease) to reduce the risk of stroke, myocardial infarction, heart failure, and cardiovascular death ("Major outcomes," 2002; Hunt et al., 2001; Lewington, Clark, & Qizilbash, 2002; Neal et al., 2000; Ogden et al., 2000). (Level of Evidence: B)
  2. Beta adrenergic–blocking drugs should be administered to all patients with Marfan syndrome and aortic aneurysm to reduce the rate of aortic dilatation unless contraindicated (Shores et al., 1994). (Level of Evidence: B)

Class IIa

  1. For patients with thoracic aortic aneurysm, it is reasonable to reduce blood pressure with beta blockers and angiotensin-converting enzyme inhibitors (Ahimastos et al., 2007) or angiotensin receptor blockers (Brooke et al., 2008; Mochizuki et al., 2007) to the lowest point patients can tolerate without adverse effects (Shores et al., 1994; Genoni et al., 2001; Ladouceur et al., 2007). (Level of Evidence: B)
  2. An angiotensin receptor blocker (losartan) is reasonable for patients with Marfan syndrome, to reduce the rate of aortic dilatation unless contraindicated (Brooke et al., 2008; Lacro et al., 2007). (Level of Evidence: B)

Recommendation for Dyslipidemia

Class IIa

  1. Treatment with a statin to achieve a target low density lipoprotein (LDL) cholesterol of less than 70 mg/dL is reasonable for patients with a coronary heart disease risk equivalent such as noncoronary atherosclerotic disease, atherosclerotic aortic aneurysm, and coexistent coronary heart disease at high risk for coronary ischemic events (Evans et al., 2007; Leurs et al., 2006; Kurzencwyg et al., 2006; Yilmaz et al., 2004). (Level of Evidence: A)

Recommendation for Smoking Cessation

Class I

  1. Smoking cessation and avoidance of exposure to environmental tobacco smoke at work and home are recommended. Follow-up, referral to special programs, and/or pharmacotherapy (including nicotine replacement, bupropion, or varenicline) is useful, as is adopting a stepwise strategy aimed at smoking cessation (the 5 A's are Ask, Advise, Assess, Assist, and Arrange) (Anderson et al., 2007; Ockene & Miller, 1997; Daly et al., 1983; Agency for Health Care Policy and Research (AHCPR), 1996). (Level of Evidence: B)

Surgical and Endovascular Treatment by Location of Disease

Ascending Aorta and Aortic Sinuses

Recommendations for Asymptomatic Patients with Ascending Aortic Aneurysm

Class I

  1. Asymptomatic patients with degenerative thoracic aneurysm, chronic aortic dissection, intramural hematoma, penetrating atherosclerotic ulcer, mycotic aneurysm, or pseudoaneurysm, who are otherwise suitable candidates and for whom the ascending aorta or aortic sinus diameter is 5.5 cm or greater, should be evaluated for surgical repair (Svensson et al., 2008). (Level of Evidence: C)
  2. Patients with Marfan syndrome or other genetically mediated disorders (vascular Ehlers-Danlos syndrome, Turner syndrome, bicuspid aortic valve, or familial thoracic aortic aneurysm and dissection) should undergo elective operation at smaller diameters (4.0 to 5.0 cm depending on the condition; see Section 5 in the original guideline document) to avoid acute dissection or rupture (Gott et al., 1999; Boissonnas et al., 2009; Svensson et al., "Relationship," 2003; Svensson et al., 2008; Kouchoukos & Dougenis, 1997; Elefteriades, 2002; Tzemos et al., 2008; Vallely, Semsarian, & Bannon, 2008). (Level of Evidence: C)
  3. Patients with a growth rate of more than 0.5 cm/y in an aorta that is less than 5.5 cm in diameter should be considered for operation. (Level of Evidence: C)
  4. Patients undergoing aortic valve repair or replacement and who have an ascending aorta or aortic root of greater than 4.5 cm should be considered for concomitant repair of the aortic root or replacement of the ascending aorta. (Level of Evidence: C)

Class IIa

  1. Elective aortic replacement is reasonable for patients with Marfan syndrome, other genetic diseases, or bicuspid aortic valves, when the ratio of maximal ascending or aortic root area (πr2) in cm2 divided by the patient's height in meters exceeds 10 (Svensson & Khitin, 2002; Svensson et al., "Relationship," 2003). (Level of Evidence: C)
  2. It is reasonable for patients with Loeys-Dietz syndrome or a confirmed TGFBR1 or TGFBR2 mutation to undergo aortic repair when the aortic diameter reaches 4.2 cm or greater by transesophageal echocardiogram (internal diameter) or 4.4 to 4.6 cm or greater by computed tomographic imaging and/or magnetic resonance imaging (external diameter) (Loeys et al., 2006). (Level of Evidence: C)

Recommendation for Symptomatic Patients with Thoracic Aortic Aneurysm

Class I

  1. Patients with symptoms suggestive of expansion of a thoracic aneurysm should be evaluated for prompt surgical intervention unless life expectancy from comorbid conditions is limited or quality of life is substantially impaired. (Level of Evidence: C)

Recommendations for Open Surgery for Ascending Aortic Aneurysm

Class I

  1. Separate valve and ascending aortic replacement are recommended in patients without significant aortic root dilatation, in elderly patients, or in young patients with minimal dilatation who have aortic valve disease. (Level of Evidence: C)
  2. Patients with Marfan, Loeys-Dietz, and Ehlers-Danlos syndromes and other patients with dilatation of the aortic root and sinuses of Valsalva should undergo excision of the sinuses in combination with a modified David reimplantation operation if technically feasible or, if not, root replacement with valved graft conduit (Vaughan et al., 2001; Hunder et al., 1990; Abedin, Tintut, & Demer, 2004; Iribarren et al., 2000; Chiu et al., 2006). (Level of Evidence: B)

Recommendations for Aortic Arch Aneurysms

Class IIa

  1. For thoracic aortic aneurysms also involving the proximal aortic arch, partial arch replacement together with ascending aorta repair using right subclavian/axillary artery inflow and hypothermic circulatory arrest is reasonable (Crawford et al., 1992; Svensson et al., "Does the arterial," 2004; Svensson et al., 1990). (Level of Evidence: B)
  2. Replacement of the entire aortic arch is reasonable for acute dissection when the arch is aneurysmal or there is extensive aortic arch destruction and leakage (Crawford et al., 1992; Svensson et al., 1990). (Level of Evidence: B)
  3. Replacement of the entire aortic arch is reasonable for aneurysms of the entire arch, for chronic dissection when the arch is enlarged, and for distal arch aneurysms that also involve the proximal descending thoracic aorta, usually with the elephant trunk procedure (Greenberg et al., 2005; Svensson, 2005; Svensson et al., "Elephant trunk procedure," 2004). (Level of Evidence: B)
  4. For patients with low operative risk in whom an isolated degenerative or atherosclerotic aneurysm of the aortic arch is present, operative treatment is reasonable for asymptomatic patients when the diameter of the arch exceeds 5.5 cm (Coady et al., 1997). (Level of Evidence: B)
  5. For patients with isolated aortic arch aneurysms less than 4.0 cm in diameter, it is reasonable to reimage using computed tomographic imaging or magnetic resonance imaging, at 12-month intervals, to detect enlargement of the aneurysm. (Level of Evidence: C)
  6. For patients with isolated aortic arch aneurysms 4.0 cm or greater in diameter, it is reasonable to reimage using computed tomographic imaging or magnetic resonance imaging, at 6-month intervals, to detect enlargement of the aneurysm. (Level of Evidence: C)

Descending Thoracic Aorta and Thoracoabdominal Aorta

Recommendations for Descending Thoracic Aorta and Thoracoabdominal Aortic Aneurysms

Class I

  1. For patients with chronic dissection, particularly if associated with a connective tissue disorder, but without significant comorbid disease, and a descending thoracic aortic diameter exceeding 5.5 cm, open repair is recommended (Svensson et al., 2008; Svensson et al., "Variables," 1993; Estrera et al., 2001). (Level of Evidence: B)
  2. For patients with degenerative or traumatic aneurysms of the descending thoracic aorta exceeding 5.5 cm, saccular aneurysms, or postoperative pseudoaneurysms, endovascular stent grafting should be strongly considered when feasible (Svensson et al., 2008; Matsumura et al., 2008). (Level of Evidence: B)
  3. For patients with thoracoabdominal aneurysms, in whom endovascular stent graft options are limited and surgical morbidity is elevated, elective surgery is recommended if the aortic diameter exceeds 6.0 cm, or less if a connective tissue disorder such as Marfan or Loeys-Dietz syndrome is present (Svensson et al., 2008). (Level of Evidence: C)
  4. For patients with thoracoabdominal aneurysms and with end-organ ischemia or significant stenosis from atherosclerotic visceral artery disease, an additional revascularization procedure is recommended (Svensson et al., 1992). (Level of Evidence: B)

Special Considerations in Pregnant Patients with Aortic Disease

Counseling and Management of Chronic Aortic Diseases in Pregnancy

Recommendations for Counseling and Management of Chronic Aortic Diseases in Pregnancy

Class I

  1. Women with Marfan syndrome and aortic dilatation, as well as patients without Marfan syndrome who have known aortic disease, should be counseled about the risk of aortic dissection as well as the heritable nature of the disease prior to pregnancy (Pearson et al., 2008; Milewicz, Dietz, & Miller, 2005). (Level of Evidence: C)
  2. For pregnant women with known thoracic aortic dilatation or a familial or genetic predisposition for aortic dissection, strict blood pressure control, specifically to prevent Stage II hypertension, is recommended. (Level of Evidence: C)
  3. For all pregnant women with known aortic root or ascending aortic dilatation, monthly or bimonthly echocardiographic measurements of the ascending aortic dimensions are recommended to detect aortic expansion until birth. (Level of Evidence: C)
  4. For imaging of pregnant women with aortic arch, descending, or abdominal aortic dilatation, magnetic resonance imaging (without gadolinium) is recommended over computed tomographic imaging to avoid exposing both the mother and fetus to ionizing radiation. Transesophageal echocardiogram is an option for imaging of the thoracic aorta. (Level of Evidence: C)
  5. Pregnant women with aortic aneurysms should be delivered where cardiothoracic surgery is available. (Level of Evidence: C)

Class IIa

  1. Fetal delivery via cesarean section is reasonable for patients with significant aortic enlargement, dissection, or severe aortic valve regurgitation (Milewicz, Dietz, & Miller, 2005). (Level of Evidence: C)

Class IIb

  1. If progressive aortic dilatation and/or advancing aortic valve regurgitation are documented, prophylactic surgery may be considered (Immer et al., 2003). (Level of Evidence: C)

Aortic Arch and Thoracic Aortic Atheroma and Atheroembolic Disease

Recommendations for Aortic Arch and Thoracic Aortic Atheroma and Atheroembolic Disease

Class IIa

  1. Treatment with a statin is a reasonable option for patients with aortic arch atheroma to reduce the risk of stroke (Tunick et al., 2002). (Level of Evidence: C)

Class IIb

  1. Oral anticoagulation therapy with warfarin (international normalized ratio [INR] 2.0 to 3.0) or antiplatelet therapy may be considered in stroke patients with aortic arch atheroma 4.0 mm or greater to prevent recurrent stroke. (Level of Evidence: C)

Perioperative Care for Open Surgical and Endovascular Thoracic Aortic Repairs

Recommendations for Preoperative Evaluation

Class I

  1. In preparation for surgery, imaging studies adequate to establish the extent of disease and the potential limits of the planned procedure are recommended. (Level of Evidence: C)
  2. Patients with thoracic aortic disease requiring a surgical or catheter-based intervention who have symptoms or other findings of myocardial ischemia should undergo additional studies to determine the presence of significant coronary artery disease. (Level of Evidence: C)
  3. Patients with unstable coronary syndromes and significant coronary artery disease should undergo revascularization prior to or at the time of thoracic aortic surgery or endovascular intervention with percutaneous coronary intervention or concomitant coronary artery bypass graft surgery. (Level of Evidence: C)

Class IIa

  1. Additional testing is reasonable to quantitate the patient's comorbid states and develop a risk profile. These may include pulmonary function tests, cardiac catheterization, aortography, 24-hour Holter monitoring, noninvasive carotid artery screening, brain imaging, echocardiography, and neurocognitive testing. (Level of Evidence: C)
  2. For patients who are to undergo surgery for ascending or arch aortic disease, and who have clinically stable, but significant (flow limiting), coronary artery disease, it is reasonable to perform concomitant coronary artery bypass graft surgery. (Level of Evidence: C)

Class IIb

  1. For patients who are to undergo surgery or endovascular intervention for descending thoracic aortic disease, and who have clinically stable, but significant (flow limiting), coronary artery disease, the benefits of coronary revascularization are not well established (Boden et al., 2007; McFalls et al., 2004; Poldermans et al., 2007). (Level of Evidence: B)

Recommendations for Choice of Anesthetic and Monitoring Techniques

Class I

  1. The choice of anesthetic techniques and agents and patient monitoring techniques should be tailored to individual patient needs to facilitate surgical and perfusion techniques and the monitoring of hemodynamics and organ function. (Level of Evidence: C)

Class IIa

  1. Transesophageal echocardiography is reasonable in all open surgical repairs of the thoracic aorta, unless there are specific contraindications to its use. Transesophageal echocardiography is reasonable in endovascular thoracic aortic procedures for monitoring, procedural guidance, and/or endovascular graft leak detection ("Practice guidelines," 1996; Fattori et al., 2000; Abe et al., 2000). (Level of Evidence: B)
  2. Motor or somatosensory evoked potential monitoring can be useful when the data will help to guide therapy. It is reasonable to base the decision to use neurophysiologic monitoring on individual patient needs, institutional resources, the urgency of the procedure, and the surgical and perfusion techniques to be employed in the open or endovascular thoracic aortic repair (Dong, MacDonald, & Janusz, 2002; Meylaerts et al., 1999). (Level of Evidence: B)

Class III

  1. Regional anesthetic techniques are not recommended in patients at risk of neuraxial hematoma formation due to thienopyridine antiplatelet therapy, low-molecular-weight heparins, or clinically significant anticoagulation (Horlocker et al., 2003). (Level of Evidence: C)
  2. Routinely changing double-lumen endotracheal (endobronchial) tubes to single-lumen tubes at the end of surgical procedures complicated by significant upper airway edema or hemorrhage is not recommended. (Level of Evidence: C)

Recommendation for Transfusion Management and Anticoagulation in Thoracic Aortic Surgery

Class IIa

  1. An algorithmic approach to transfusion, antifibrinolytic, and anticoagulation management is reasonable to use in both open and endovascular thoracic aortic repairs during the perioperative period. Institutional variations in coagulation testing capability and availability of transfusion products and other prothrombotic and antithrombotic agents are important considerations in defining such an approach (Society of Thoracic Surgeons Blood Conservation Guideline Task Force et al., 2007). (Level of Evidence: C)

Organ Protection

Recommendations for Brain Protection During Ascending Aortic and Transverse Aortic Arch Surgery

Class I

  1. A brain protection strategy to prevent stroke and preserve cognitive function should be a key element of the surgical, anesthetic, and perfusion techniques used to accomplish repairs of the ascending aorta and transverse aortic arch (Akashi et al., 2000; Ehrlich et al., 1999; Di Eusanio et al., "Deep hypothermic," 2003; Hagl et al., 2001; Reich et al., 1999; Reich et al., 2001; Usui et al., 1999). (Level of Evidence: B)

Class IIa

  1. Deep hypothermic circulatory arrest, selective antegrade brain perfusion, and retrograde brain perfusion are techniques that alone or in combination are reasonable to minimize brain injury during surgical repairs of the ascending aorta and transverse aortic arch. Institutional experience is an important factor in selecting these techniques (Svensson, Nadolny, & Kimmel, 2002; Svensson et al., "Deep hypothermia," 1993; Kunihara et al., 2005; Schepens et al., 2002; Deeb et al., 1999; Ehrlich et al., 2003; Fleck et al., 2003; Moshkovitz et al., 1998; Okita et al., 1998; Ueda et al., 1999; Wong & Bonser, 1999; Di Eusanio et al., "Brain protection," 2003; Di Eusanio et al., 2002; Di Eusanio et al., "Surgery," 2003; Kazui et al., 2002; Kazui et al., 2007; Numata et al., 2003; Sasaki et al., 2007; Strauch et al., 2004; Kamiya et al., 2007; Matalanis, Hata, & Buxton, 2003; Okita et al., 2001; Zierer et al., 2005; Okita et al., 1999). (Level of Evidence: B)

Class III

  1. Perioperative brain hyperthermia is not recommended in repairs of the ascending aortic and transverse aortic arch as it is probably injurious to the brain (Grigore et al., 2002; Grocott et al., 2002; Bar-Yosef et al., 2004). (Level of Evidence: B)

Recommendations for Spinal Cord Protection During Descending Aortic Open Surgical and Endovascular Repairs

Class I

  1. Cerebrospinal fluid drainage is recommended as a spinal cord protective strategy in open and endovascular thoracic aortic repair for patients at high risk of spinal cord ischemic injury (Coselli et al., 2002; Safi et al., 2003; Khan & Stansby, 2004). (Level of Evidence: B)

Class IIa

  1. Spinal cord perfusion pressure optimization using techniques, such as proximal aortic pressure maintenance and distal aortic perfusion, is reasonable as an integral part of the surgical, anesthetic, and perfusion strategy in open and endovascular thoracic aortic repair patients at high risk of spinal cord ischemic injury. Institutional experience is an important factor in selecting these techniques (Estrera et al., 2005; Svensson et al., "Variables," 1993; Safi et al., 1996; Hollier et al., 1992). (Level of Evidence: B)
  2. Moderate systemic hypothermia is reasonable for protection of the spinal cord during open repairs of the descending thoracic aorta (Svensson et al., "Systemic temperature," 2003). (Level of Evidence: B)

Class IIb

  1. Adjunctive techniques to increase the tolerance of the spinal cord to impaired perfusion may be considered during open and endovascular thoracic aortic repair for patients at high risk of spinal cord injury. These include distal perfusion, epidural irrigation with hypothermic solutions, high-dose systemic glucocorticoids, osmotic diuresis with mannitol, intrathecal papaverine, and cellular metabolic suppression with anesthetic agents (Cambria et al., 2000; Hollier et al., 1992; Cambria et al., 1997; Woloszyn et al., 1990). (Level of Evidence: B)
  2. Neurophysiological monitoring of the spinal cord (somatosensory evoked potentials or motor evoked potentials) may be considered as a strategy to detect spinal cord ischemia and to guide reimplantation of intercostal arteries and/or hemodynamic optimization to prevent or treat spinal cord ischemia (Schurink et al., 2007; Dong, MacDonald, & Janusz, 2002; Ogino et al., 2006; Guerit et al., 1999). (Level of Evidence: B)

Recommendations for Renal Protection During Descending Aortic Open Surgical and Endovascular Repairs

Class IIb

  1. Preoperative hydration and intraoperative mannitol administration may be reasonable strategies for preservation of renal function in open repairs of the descending aorta. (Level of Evidence: C)
  2. During thoracoabdominal or descending aortic repairs with exposure of the renal arteries, renal protection by either cold crystalloid or blood perfusion may be considered (Svensson et al., "Appraisal," 1989; Jacobs et al., 1997; Koksoy et al., 2002). (Level of Evidence: B)

Class III

  1. Furosemide, mannitol, or dopamine should not be given solely for the purpose of renal protection in descending aortic repairs (Perdue et al., 1998; Hager, Betschart, & Krapf, 1996). (Level of Evidence: B)

Postprocedural Care

Postoperative Risk Factor Management

Recommendations for Surveillance of Thoracic Aortic Disease or Previously Repaired Patients

Class IIa

  1. Computed tomographic imaging or magnetic resonance imaging of the thoracic aorta is reasonable after a Type A or B aortic dissection or after prophylactic repair of the aortic root/ascending aorta (Pearson et al., 2008). (Level of Evidence: C)
  2. Computed tomographic imaging or magnetic resonance imaging of the aorta is reasonable at 1, 3, 6, and 12 months postdissection and, if stable, annually thereafter so that any threatening enlargement can be detected in a timely fashion. (Level of Evidence: C)
  3. When following patients with imaging, utilization of the same modality at the same institution is reasonable, so that similar images of matching anatomic segments can be compared side by side. (Level of Evidence: C)
  4. If a thoracic aortic aneurysm is only moderate in size and remains relatively stable over time, magnetic resonance imaging instead of computed tomographic imaging is reasonable to minimize the patient's radiation exposure. (Level of Evidence: C)
  5. Surveillance imaging similar to classic aortic dissection is reasonable in patients with intramural hematoma. (Level of Evidence: C)

Long-Term Issues

Recommendation for Employment and Lifestyle in Patients with Thoracic Aortic Disease

Class IIa

  1. For patients with a current thoracic aortic aneurysm or dissection, or previously repaired aortic dissection, employment and lifestyle restrictions are reasonable, including the avoidance of strenuous lifting, pushing, or straining that would require a Valsalva maneuver. (Level of Evidence: C)

Institutional/Hospital Quality Concerns

Recommendations for Quality Assessment and Improvement for Thoracic Aortic Disease

Class I

  1. Hospitals that provide regional care for patients with acute sequelae of thoracic aortic disease (e.g., procedures for thoracic aortic dissection and rupture) should participate in standardized quality assessment and improvement activities, including thoracic aortic disease registries. Such activities should include periodic measurement and regional/national interfacility comparisons of thoracic aortic disease-related procedural volumes, complications, and risk-adjusted mortality rates. (Level of Evidence: C)
  2. Hospitals that provide regional care for patients with acute sequelae of thoracic aortic disease (e.g., procedures for thoracic aortic dissection and rupture) should facilitate and coordinate standardized quality assessment and improvement activities with transferring facilities and emergency medical services teams. Such activities might include:
    1. Cooperative joint facility meetings to discuss opportunities for quality improvement and
    2. Interfacility and emergency medical services team comparisons of pretransfer care based on available outcome data and future performance measures developed in accordance with this guideline. (Level of Evidence: C)

Definitions:

Applying Classification of Recommendations and Level of Evidence

  Size of Treatment Effect
  CLASS I

Benefit >>> Risk

Procedure/Treatment
SHOULD be performed/ administered
CLASS IIa

Benefit >> Risk
Additional studies with focused objectives needed


IT IS REASONABLE to perform procedure/administer treatment
CLASS IIb

Benefit ≥ Risk
Additional studies with broad objectives needed; additional registry data would be helpful


Procedure/Treatment
MAY BE CONSIDERED
CLASS III No Benefit
or Class III Harm
  Procedure/Test Treatment
COR III:
No Benefit
Not helpful No proven benefit
COR III:
Harm
Excess Cost without Benefit or Harmful Harmful to Patients
Estimate of Certainty (Precision) of Treatment Effect LEVEL A

Multiple populations evaluated*

Data derived from multiple randomized clinical trials or meta-analyses
  • Recommendation that procedure or treatment is useful/effective
  • Sufficient evidence from multiple randomized trials or meta-analyses
  • Recommendation in favor of treatment or procedure being useful/effective
  • Some conflicting evidence from multiple randomized trials or meta-analyses
  • Recommendation's usefulness/efficacy less well established
  • Greater conflicting evidence from multiple randomized trials or meta-analyses
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Sufficient evidence from multiple randomized trials or meta-analyses
LEVEL B

Limited populations evaluated*

Data derived from a single randomized clinical trials or nonrandomized studies
  • Recommendation that procedure or treatment is useful/effective
  • Evidence from single randomized trial or nonrandomized studies
  • Recommendation in favor of treatment or procedure being useful/effective
  • Some conflicting evidence from single randomized trial or nonrandomized studies
  • Recommendation's usefulness/efficacy less well established
  • Greater conflicting evidence from single randomized trial or nonrandomized studies
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Evidence from single randomized trial or nonrandomized studies
LEVEL C

Very limited populations evaluated*

Only consensus opinion of experts, case studies or standard of care
  • Recommendation that procedure or treatment is useful/effective
  • Only expert opinion, case studies, or standard of care
  • Recommendation in favor of treatment or procedure being useful/effective
  • Only diverging expert opinion, case studies, or standard of care
  • Recommendation's usefulness/efficacy less well established
  • Only diverging expert opinion, case studies, or standard of care
  • Recommendation that procedure or treatment is not useful/effective and may be harmful
  • Only expert opinion, case studies, or standard of care

*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.

Clinical Algorithm(s)

The original guideline document contains the following clinical algorithms:

  • Acute surgical management pathway for aortic dissection (AoD)
  • AoD evaluation pathway
  • Acute AoD management pathway
  • Ascending aortic aneurysm of degenerative etiology
  • Ascending aortic aneurysms associated with genetic disorder

Evidence Supporting the Recommendations

References Supporting the Recommendations
Type of Evidence Supporting the Recommendations

The type of supporting evidence is identified and graded for each recommendation (see the "Major Recommendations" field).

The writing committee recognized that the evidence base for this guideline is less robust in terms of randomized clinical trials than prior American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines, particularly those focused on coronary artery disease and heart failure. Much of the evidence base for this topic consists of cohort studies and retrospective reviews, which largely emanate from centers with a specialized interest in specific types of thoracic aortic disease.

Benefits/Harms of Implementing the Guideline Recommendations

Potential Benefits

Increased early recognition and prompt treatment and/or referral for various thoracic aortic diseases by all healthcare professionals

Potential Harms
  • Radiation exposure should be minimized. The risk of radiation-induced malignancy is the greatest in neonates, children, and young adults. Generally, above the age of 30 to 35 years, the probability of radiation-induced malignancy decreases substantially.
  • There is concern about the use of warfarin in patients with aortic atheroma because of the theoretical risk of plaque hemorrhage resulting in atheroemboli syndrome (i.e., blue toes, renal failure, intestinal infarction). Anticoagulation has been associated with worsening, as well as improvement of an aortic thrombus on anticoagulation in a patient with the atheroemboli syndrome. Cholesterol emboli have been documented on skin, muscle, and renal biopsy samples in patients with aortic arch atheroma seen on transesophageal echocardiography. However, the risk of clinical atheroemboli syndrome during warfarin therapy in such patients appears to be low (only 1 episode in 134 patients according to the Stroke Prevention in Atrial Fibrillation [SPAF] trial).
  • Endovascular approach for descending thoracic aorta thoracoabdominal aortic aneurysms
    • Intervention (endovascular stent graft or open surgical graft replacement) for a descending aneurysm has real risks of mortality and morbidity, including the risk of spinal cord ischemic injury. All physicians should work collaboratively among specialties during the initial decision-making steps to determine via consensus whether a particular patient's pathology, risk factors, and projected natural history if treated medically justify an intervention on the descending thoracic aorta, either a stent graft or an open procedure.
    • Because aneurysmal disease can involve any portion of the aorta, organ preservation (including brain, spinal cord, upper and lower extremities, intestines, and kidneys) during repair of either aneurysmal disease or dissection is an important part of the implant procedure.
    • See Section 9.2.2.3.4, "Periprocedural Complications of Endograft Procedures," in the original guideline document for specific complications.
    • A complication unique to endograft treatment of acute Type B dissections is conversion of the dissection to a retrograde Type A dissection, creating a surgical emergency. Until and unless this life threatening complication is understood and eliminated, endograft treatment of acute Type B dissections should be undertaken at institutions with a team qualified to perform open aortic surgery.
  • See Section 14.6 in the original guideline document for complications of open surgical approaches.
  • See Section 14.7 in the original guideline document for mortality risk for thoracic aortic surgery.

Contraindications

Contraindications
  • Magnetic resonance imaging is contraindicated in patients with claustrophobia, metallic implants, or pacemakers.
  • Potential contraindication to beta blockade include: asthma, congestive heart failure, or chronic obstructive pulmonary disease.
  • Contraindications to endovascular grafting procedures:
    • Absence of suitable "landing zones" above and below the aneurysm (usually 2 to 3 cm of normal diameter aorta without circumferential thrombus)
    • A width of the aorta at the "landing zones" that exceeds the recommended width for the largest available endovascular grafts (generally 10% to 15% larger than the width of the aorta)
    • Lack of vascular access sites to insert the relatively large-bore sheaths that are necessary for deployment of the grafts
    • Severe atherosclerosis and intraluminal thrombus of the aorta may increase the risk of embolic stroke during manipulation of guidewires and catheters and represents a relative contraindication.
    • Younger patient age may be a relative contraindication to endografting.
  • Both angiotensin-converting enzyme inhibitors and angiotensin receptor blockers are contraindicated during pregnancy.

Qualifying Statements

Qualifying Statements
  • The American College of Cardiology Foundation/American Heart Association (ACCF/AHA) practice guidelines are intended to assist healthcare providers in clinical decision making by describing a range of generally acceptable approaches for diagnosis, management, and prevention of specific diseases or conditions. Clinicians should consider the quality and availability of expertise in the area where care is provided. These guidelines attempt to define practices that meet the needs of most patients in most circumstances. The recommendations reflect a consensus after a thorough review of the available current scientific evidence and are intended to improve patient care. The Task Force recognizes that situations arise where additional data are needed to better inform patient care; these areas will be identified within each respective guideline when appropriate.
  • Patient adherence to prescribed and agreed upon medical regimens and lifestyles is an important aspect of treatment. Prescribed courses of treatment in accordance with these recommendations are effective only if they are followed. Because lack of patient understanding and adherence may adversely affect outcomes, physicians and other healthcare providers should make every effort to engage the patient's active participation in prescribed medical regimens and lifestyles.
  • If these guidelines are used as the basis for regulatory or payer decisions, the goal should be improvement in quality of care and aligned with the patient's best interest. The ultimate judgment regarding care of a particular patient must be made by the healthcare provider and the patient in light of all of the circumstances presented by that patient. Consequently, there are circumstances in which deviations from these guidelines are appropriate.

Implementation of the Guideline

Description of Implementation Strategy

An implementation strategy was not provided.

Implementation Tools
Clinical Algorithm
Pocket Guide/Reference Cards
Quick Reference Guides/Physician Guides
Slide Presentation
For information about availability, see the Availability of Companion Documents and Patient Resources fields below.

Institute of Medicine (IOM) National Healthcare Quality Report Categories

IOM Care Need
Getting Better
Living with Illness
Staying Healthy
IOM Domain
Effectiveness
Patient-centeredness

Identifying Information and Availability

Bibliographic Source(s)
Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM, American College of Cardiology Foundation/American Heart Association Task Force [trunc], American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, Society for Vascular Medicine. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. A report of the American College of Cardiology Foundation/American Heart Association Task Force [trunc]. J Am Coll Cardiol. 2010 Apr 6;55(14):e27-e129. [827 references] PubMed External Web Site Policy
Adaptation

Not applicable: The guideline was not adapted from another source.

Date Released
2010 Apr 6
Guideline Developer(s)
American Association for Thoracic Surgery - Medical Specialty Society
American College of Cardiology Foundation - Medical Specialty Society
American College of Radiology - Medical Specialty Society
American Heart Association - Professional Association
American Stroke Association - Disease Specific Society
Society for Cardiovascular Angiography and Interventions - Medical Specialty Society
Society for Vascular Medicine - Medical Specialty Society
Society of Cardiovascular Anesthesiologists - Medical Specialty Society
Society of Interventional Radiology - Medical Specialty Society
Society of Thoracic Surgeons - Medical Specialty Society
Source(s) of Funding

American College of Cardiology Foundation

Guideline Committee

American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Task Force

Composition of Group That Authored the Guideline

Writing Group Members: Loren F. Hiratzka, MD (Chair); George L. Bakris, MD; Joshua A. Beckman, MD, MS; Robert M. Bersin, 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

ACCF/AHA Task Force Members: Alice K. Jacobs, MD, FACC, FAHA (Chair 2009–2011); Sidney C. Smith, Jr, MD, FACC, FAHA (Immediate Past Chair 2006–2008)*; Jeffery L. Anderson, MD, FACC, FAHA (Chair-Elect); Cynthia D. Adams, MSN, PhD, FAHA*; Christopher E. Buller, MD, FACC; Mark A. Creager, MD, FACC, FAHA; Steven M. Ettinger, MD, FACC; Robert A. Guyton, MD, FACC, FAHA; Jonathan L. Halperin, MD, FACC, FAHA; Sharon A. Hunt, MD, FACC, FAHA*; Harlan M. Krumholz, MD, FACC, FAHA*; Frederick G. Kushner, MD, FACC, FAHA; Bruce W. Lytle, MD, FACC, FAHA*; Rick Nishimura, MD, FACC, FAHA*; Richard L. Page, MD, FACC, FAHA*; Barbara Riegel, DNSc, RN, FAHA; William G. Stevenson, MD, FACC, FAHA; Lynn G. Tarkington, RN; Clyde W. Yancy, MD, FACC, FAHA

*Former Task Force member during this writing effort

Financial Disclosures/Conflicts of Interest

The American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Task Force on Practice Guidelines makes every effort to avoid actual, potential, or perceived conflicts of interest that may arise as a result of industry relationships or personal interests among the writing committee. Specifically, all members of the writing committee, as well as peer reviewers of the document, are asked to disclose all current relationships and those 24 months prior to initiation of the writing effort that may be perceived as relevant. All guideline recommendations require a confidential vote by the writing committee and must be approved by a consensus of the members voting. Members who were recused from voting are noted on the title page of this document. Members must recuse themselves from voting on any recommendation where their relationships with industry (RWI) apply. If a writing committee member develops a new relationship with industry during his/her tenure, he/she is required to notify guideline staff in writing. These statements are reviewed by the Task Force on Practice Guidelines and all members during each conference call and/or meeting of the writing committee, updated as changes occur, and ultimately published as an appendix to the document. For detailed information regarding guideline policies and procedures, please refer to the methodology manual for ACCF/AHA Guideline Writing Committees (see the "Availability of Companion Documents" field).

RWI and other entities pertinent to this guideline for authors and peer reviewers are disclosed in Appendixes 1 and 2 of the original guideline document, respectively. Disclosure information for the ACCF/AHA Task Force on Practice Guidelines is also available is also available from the American College of Cardiology Web site External Web Site Policy.

Guideline Endorser(s)
North American Society for Cardiovascular Imaging - Professional Association
Guideline Status

This is the current release of the guideline.

Guideline Availability

Electronic copies: Available from the Journal of the American College of Cardiology (ACC) Web site External Web Site Policy and from the American Heart Association (AHA) Web site External Web Site Policy.

Print copies: Available from the ACC, 2400 N Street NW, Washington DC, 20037; (800) 253-4636 (US only).

Availability of Companion Documents

The following are available:

  • Methodology manual and policies from the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Task Force on Practice Guidelines. 2010 Jun. 88 p. American College of Cardiology Foundation and American Heart Association, Inc. Electronic copies: Available in PDF from the American College of Cardiology (ACC) Web site External Web Site Policy.
  • 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. 2010. 36 p. Electronic copies: Available in Portable Document Format (PDF) from the Journal of the American College of Cardiology (JACC) Web site External Web Site Policy.
  • ACCF/AHA pocket guideline. Guidelines for the diagnosis and management of patients with thoracic aortic disease. 2010. 69 p. Electronic copies: Available in PDF from the JACC Web site External Web Site Policy.
  • 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. Slide set. Electronic copies: Available to subscribers from the JACC Web site External Web Site Policy.
  • Technique parameters and anatomical coverage for thoracic aortic computed tomography studies. Supplemental content. Electronic copies: Available in PDF from the JACC Web site External Web Site Policy.
Patient Resources

None available

NGC Status

This summary was completed by ECRI Institute on August 7, 2013. This summary was updated by ECRI Institute on July 10, 2014 to insert a message from the guideline developers on the use of beta blockers in perioperative care.

Copyright Statement

This NGC summary is based on the original guideline, which is subject to the guideline developer's copyright restrictions as follows:

Copyright to the original guideline is owned by the American College of Cardiology Foundation (ACCF) and the American Heart Association, Inc. (AHA). NGC users are free to download a single copy for personal use. Reproduction without permission of the ACC/AHA guidelines is prohibited. Permissions requests should be directed to copyright_permissions@acc.org.

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