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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Initial observation


The initial observation of the patient may reveal physical features characteristic of a genetic syndrome associated with an increased risk for aortic dissections. These include:

  • Marfan syndrome
    • Facial features such as long, narrow face, underdeveloped cheekbones, deep set eyes, receding lower jaw, downward slanting eyes
    • Ectopia lentis (dislocated lens of the eye)
    • High arched palate and crowding of the teeth
    • Tall stature, disproprionately long arms, legs, fingers and/or toes
    • Pectus excavatum (sunken chest) or pectus carinatum (pigeon breast)
    • Scoliosis or kyphosis
    • Flat or inwardly rotated feet, hammer toes
    • Joint hypermobility
    • History of a pneumothorax
    • Unexplained striae (skin stretch marks)
  • Loeys-Dietz syndrome
    • Facial features such as widely spaced eyes,, downward-slanting eyes, underdeveloped cheek bones, small chin and/or receding chin
    • Blue sclerae (blue tinge to the whites of the eyes)
    • Craniosynostosis (early fusion of the skull bones)
    • Cleft palate
    • Bifid (split) or broad uvula
    • Long fingers and/or toes, contractures of the fingers
    • Pectus excavatum (sunken chest) or pectus carinatum (pigeon breast)
    • Cervical-spine instability
    • Scoliosis
    • Clubfoot or skewfoot deformity
    • Joint hypermobility
    • Congenital heart defects (patent ductus arteriosus atrial or ventricular septal defect, bicuspid aortic valve)
  • Ehlers-Danlos syndrome-vascular type
    • Facial features such as thin lips and philtrum, small chin, thin nose, large and proptotic eyes
    • Thin, translucent skin with prominent veins, particularly on chest and abdomen
    • Joint hypermobility
    • Acrogeria (aged appearance to the hands and feet)
    • Clubfoot
    • History of a pneumothorax
  • Turner syndrome
    • Short stature
    • Webbed neck
    • Low hairline at the back of the neck
    • Lymphedema of the hands and feet
    • Bicuspid aortic valve, coarctation of the aorta

Chief Ccomplaint

Symptoms of aortic dissection are extremely variable, but are often described by patients as:

  • Sudden onset severe pain along middling in chest, back, or abdominal
  • Penetrating pain front to back
  • Ripping, burning, or tearing sensation
  • Migratory
  • Pain like they have never experienced before or sense of impending doom

A patient with an aortic dissection may not appear to be a “typical” chest pain patient – he or she may be younger, tall/thin, and have no known risk factors for heart disease.