Congenital Heart Disease in the Emergency Department

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Aortic Stenosis (AS)

The aortic valve leaflets are partly fused together, giving a restrictive exit from the left ventricle. There may be one to three aortic leaflets. Aortic stenosis may not be an isolated lesion.

May occur at valvular, supra-valvular or sub-valvular level.

Bicuspid aortic valve is most common type

Williams syndrome: supra-valvular stenosis

aorticstenosis

Clinical presentation

Symptoms
  • Most present with an asymptomatic murmur
  • Congestive heart failure (with severe obstruction)
  • In the neonatal period, those with critical aortic stenosis and a duct-dependent systemic circulation may present with severe heart failure leading to shock
Physical signs
  • Small volume, slow rising pulses
  • Systolic thrill (right sternal border, suprasternal notch or carotid arteries)
  • Ejection systolic murmur maximal at the upper right sternal edge radiating to the neck
  • Delayed and soft aortic second sound
  • Apical ejection click

Investigations

Chest radiograph
  • Normal or prominent left ventricle with post-stenotic dilatation of the ascending aorta
ECG
  • Left ventricular hypertrophy

Management

In children, regular clinical and echocardiographic assessment is required in order to assess when to intervene. Children with symptoms on exercise or who have a high resting pressure gradient (more than 50 mm Hg) across the aortic valve will undergo balloon valvotomy.

Most neonates and children with significant aortic valve stenosis requiring treatment in the first few years of life will eventually require aortic valve replacement. Early treatment is therefore palliative and directed towards delaying this for as long as possible.