AHA/ACC Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease (2010)

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Commentary by Reed E. Pyeritz, MD, PhD

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Risk assessment

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AoD Evaluation: risk assessment

Patients with acute AoD are subject to missed or delayed detection of this catastrophic disease state. Many present with atypical symptoms and findings, making diagnosis even more difficult. Awareness of the varied and complex nature of thoracic aortic disease presentations has been lacking, especially for acute AoD, but is critical to facilitate early diagnosis and treatment.

Risk factors, clinical presentation clues and recommendations for pretest risk assessment are presented below.

Risk factors for development of thoracic aortic dissection 

Conditions Associated with Increased Aortic Wall StressConditions Associated with Aortic Media Abnormalities
  • Hypertension, particularly if uncontrolled
  • Pheochromocytoma
  • Cocaine or other stimulant use
  • Weight-lifting or other Valsalva maneuver
  • Trauma
  • Deceleration or torsional injury (e.g., motor vehicle crash, fall)
  • Coarctation of the aorta
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 dissections
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

Recommendations for estimate of pretest risk of thoracic aortic dissection

Class 1
  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:

  2. 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.
  1. 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.
  1. 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).
 
  1. Patients presenting with sudden onset of severe chest, back, and/or abdominal pain, particularly those under the age of 40, should be questioned about a family history of a connective tissue disorder and examined for physical features of Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-Danlos syndrome, Turner syndrome, or other connective tissue disorders associated with thoracic aortic disease. (LOE: B)
 
  1. 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)
 
  1. 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)
 
  1. 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)
 
  1. 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)