questions 2

Coarctation of the Aorta

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Topic updated on 04/21/17 9:11am

Snap Shot
  • A 7-year-old-boy is seen for the first time by a primary care physician. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mm Hg. His lower extremities are slightly atrophic with a mottling appearance.
Introduction
  • A congenital heart defect characterized by narrowing of the lumen of the aorta  
    • infantile/preductal type 
      • occurs distal to the subclavian artery and proximal to the ductus arteriosus 
    • adult/postductal type 
      • occurs distal to the ductus arteriosus/ligamentum arteriosum 
    • leads to decreased flow distal to the constriction and increased flow proximal to it 
  • Risk factors include 
    • Turner syndrome
    • affects males > females
    • is associated with bicuspid aortic valve (~70%)
  • A cause of secondary HTN due to underperfusion of the kidneys
    • results in activation of renin-angiotensin system
Presentation
  • Symptoms 
    • patients present in childhood with
      • asymptomatic HTN
      • dyspnea on exertion
      • systemic hypoperfusion (shock state)
      • without cyanosis
  • Physical exam
    • shows systolic BP in the upper extremities greater than in the lower extremities
      • with weak femoral and distal pulses
    • continuous murmur over collateral vessels in the back
      • dilation of aorta and aortic valve ring resulting in regurgitation
    • lower extremity skin mottling
    • leg claudication
Evaluation
  • CXR may reveal "rib notching"  
    • due to collateral circulation through intercostals  
  • CXR also shows "3 sign" around the site of coarctation
  • Echo shows left ventricular hypertrophy



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TAG
(M1.CV.158) A 5-year-old girl presents to the physician with increased muscle cramping in her lower extremities after walking extended distances. The young girl is in the 10th percentile for height. Her past medical history is notable only for a cystic hygroma detected shortly after birth. Which of the following findings is most likely in this patient? Topic Review Topic

1. Decreased blood pressure in the upper and lower extremities
2. Barr bodies on buccal smear
3. Endocardial cushion defect
4. Inferior erosion of the ribs
5. Apparent hypertrophy of the calves

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