questions 2

Aortic Dissection

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Topic updated on 09/17/17 12:35pm

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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Qbank (1 Questions)

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(M1.CV.61) A 25-year-old male presents for a new primary-care visit. He has never been seen by a physician and reports that he has been in good health. You note a very tall, very thin male whose arm span is greater than his height. The patient reports that his father had a similar build but passed away suddenly in his 40s. You suspect a genetic disorder characterized by a defect in fibrillin-1. What is the histopathology of the most common large-artery complication of this disease? Topic Review Topic

1. Focal granulomatous inflammation with mural lymphocytes, macrophages, giant cells
2. Eosinophilic vasculitis
3. Predominant neutrophilic infiltration with fibrinoid necrosis
4. Fibrinoid necrosis of blood vessel walls, endothelial swelling, and neutrophilic infiltrate in skin lesions
5. Cystic medial degeneration

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