Updated: 9/27/2020

Coarctation of Aorta

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Snapshot
  • A neonatal boy is born without any complications to a 23-year-old mother who did not receive consistent prenatal care. The boy did very well in the first few hours of life, earning APGAR scores of 8 and 9. After 6 hours, however, he began having trouble feeding. He was tachypneic especially when feeding or when crying. His mom noticed that his lower extremities also appeared bluer than his upper extremities. An echocardiogram is scheduled immediately.
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
      • 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
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 less common but possible
    • hypertension in upper extremities
      • systolic blood pressure in the arms is > 20 mmHg 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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Questions (4)
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(M2.CV.15.4672) An 11-year-old girl presents to her pediatrician with complaints of headache and rapid leg fatigue with exercise. Her vital signs are: HR 77, BP in left arm 155/100, RR 14, SpO2 100%, T 37.0 C. On palpation, her left dorsalis pedis pulse is delayed as compared to her left radial pulse. Her lower extremities feel slightly cool. Blood pressure obtained on the left lower extremity is 120/80. Which of the following is the most likely underlying pathology?

QID: 107178
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Peripheral arterial disease

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Vasospasm

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Coarctation of the aorta

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(24/26)

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Tetralogy of Fallot

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Aortic stenosis

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M 6 C

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