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Snapshot
  • A 4-year-old girl presents to her pediatrician’s office for a well-child visit. She and her parents report no concerns except for occasional headaches. During evaluation of her vital signs, her lower extremity systolic blood pressure is found to be more than 20 mmHg less than her upper extremity systolic blood pressure.
Introduction
  • Clinical definition
    • narrowing of the aorta causing hypertension in the upper extremities relative to the lower extremities
      • post-ductal type (adult type)
        • narrowing occurs distal to the ductus arteriosum
      • pre-ductal (infantile type)
        • narrowing occurs proximal to the ductus arteriosum
  • Epidemiology
    • demographics
      • male > female
      • <10% of all congenital heart defects
    • location
      • aorta
    • risk factors
      • family history
  • Pathogenesis
    • mechanism of coarctation is unknown but thought to be due to
      • decreased blood flow across aorta in utero, which can lead to defects in the aorta
      • ectopic ductal tissue in the aorta, which can cause the aorta to be pulled inwards into a coarctation
    • because of the aortic narrowing, there is hypertension in the upper extremities
      • pulse is delayed in the lower extremities
    • collateral circulation causes intercostal arteries to enlarge
      • these arteries then erode the ribs, causing a notched appearance on radiography
  • Associated conditions
    • Turner syndrome
    • bicuspid aortic valve
    • Williams syndrome
  • Prognosis
    • some may present early in life, but others may remain asymptomatic until adulthood
    • infants may be asymptomatic until the ductus arteriosus closes
Presentation
  • Symptoms
    • headache
    • shortness of breath with exertion
    • exercise intolerance
    • fatigue
    • poor feeding in infants
  • Physical exam
    • cardiac
      • harsh systolic murmur along the left sternal border
      • may also have systolic murmur along left and right side of the chest with thrills
        • from collateral circulation over time
    • pulses
      • femoral < brachial pulses
      • bounding pulses in upper extremities and carotids
      • delay in femoral pulse compared to the radial pulse
    • differential cyanosis
      • cyanotic lower extremities
    • hypertension in upper extremities
      • systolic blood pressure in the arms are > 20 mm Hg higher than the legs
    • underdeveloped legs compared with arms
Imaging
  • Radiography
    • indication
      • to rule out any pulmonary pathologies
    • views
      • chest
    • findings
      • rib notching on the inferior surface
        • due to collateral circulation through the intercostals
      • cardiomegaly
      • increased pulmonary markings
  • Echocardiography
    • indication
      • performed as a diagnostic test
      • most specific test
    • findings
      • coarctation of aorta visualized
Studies
  • Electrocardiogram
    • findings
      • left ventricular hypertrophy
  • Making the diagnosis
    • based on clinical presentation and echocardiography
Differential
  •  Tetralogy of Fallot
    • distinguishing factor
      • tet spells (cyanosis) that are resolved when placed in knee-chest position
Treatment
  • Medical
    • prostaglandin E
      • indication
        • neonates with coarctation of the aorta
      • mechanism of action
        • maintains a patent ductus arteriosus for adequate lower extremity perfusion
    • diuretics
      • indications
        • heart failure
  • Operative
    • surgical or transcatheter repair
      • indication
        • adults and neonates after stabilization of heart failure or shock
        • children with hypertension
      • modalities
        • balloon angioplasty
        • stent placement
Complications
  • Heart failure
  • Systemic hypertension
  • Berry aneurysms leading to cerebral hemorrhage
  • Aortic rupture
  • Endocarditis

 

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