Pediatric Aortic Stenosis

• Etiology:
  — Occurs at level of the aortic valve in 60-75% of cases
  — Supravalvular stenosis is rare and almost always associated with infantile hypercalcemia (Williams syndrome)
  — Associated with bicuspid aortic valve in greater than 50% of cases
• Imaging CXR:
  — Usually normal in mild cases
  — In more severe cases over time can get left ventricular hypertrophy and then left ventricular dilation
  — Post stenotic dilation of ascending aorta is most persistent radiologic finding due to jet of blood shooting through stenotic valve and striking lateral aortic wall
  — “Left ventricular configuration” is when there is a concavity to the left mid-heart border combined with a prominent lower left heart border
• DDX:
• Complications: Left ventricular hypertrophy develops in response to increased ventricular end systolic pressure and dilation does not occur until the stenosis is complicated by ventricular decompensation
• Treatment:
• Clinical:
  — Most are asymptomatic
  — Symptoms can include chest pain and syncope
  — 13% get congestive heart failure in the first year of life, congestive heart failure rarely develops later in life

Radiology Cases of Aortic Stenosis