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Overview
 

 
Snapshot
  • A 32-year-old man with eunuchoid proportions and arachnodactyly comes to the ER complaining of sudden onset of severe substernal chest pain with severe pain radiating down his back. CXR shows a widened mediastinum.
Introduction
  • The spread of blood into an intimal tear in the wall of the aorta causing a second lumen to form
    • caused by elastic tissue degradation
    • blood enters space between intima and tunica media
    • blood can pass proximally or distally
  • Risk factors 
    • hypertension
      • due to damage to endothelial cell layer predisposing to tear
    • trauma
    • coarctation of the aorta
    • syphilis
      • due to vasculitis and occlusion of vasa vasorum 
    • Ehlers-Danlos syndrome
    • Marfan's Syndrome  (due to cystic medial degeneration resulting from defect in fibrillin-1)
    • pregnancy
      • due to ↑ plasma volume
    • copper deficiency 
      • due to weak collagen without copper-dependent enzymatic cross-linking
  • Epidemiology
    • classically a 40-60 year-old man with hypertension
Presentation
  • Symptoms
    • classical presentation
      • acute onset of severe tearing anterior chest pain radiating to the back
      • asymmetric upper extremity blood pressures
        • due to compression of subclavian artery unilaterally
    • in severe cases
      • syncope
      • decreased pulse
      • shock
      • rupture into pericardial sac, pleural cavity, or peritoneal cavity
        • cardiac tamponade is the most common cause of death
  • Physical Exam
    • asymmetric upper extremity pulses
    • diastolic murmur secondary to aortic regurgitation
      • dilation of the aorta stretches the aortic ring with widening of the aortic root
Evaluation
  • EKG
    • may show LVH and ischemic changes
  • Imaging
    • CXR shows widened superior mediastinum
    • CT with contrast reveals true (arrow) and false lumen  

 

 

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