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Congenital aortic stenosis accounts for about 5% of all causes of CHD and is the commonest cause of left ventricular outflow obstruction. It is due to thickening of the aortic valves, although subvalvular (subaortic) stenosis is also an important form of obstruction. Congenital aortic stenosis is more common in boys (3:1). A supravalvular form of aortic stenosis is also rec-ognized, which may be sporadic or familial. Supravalvular aortic stenosis is also associated with Williams syndrome.
Are dependent on the severity of obstruction and age at presentation. Mild stenosis is usually asymptomatic and found on routine examination. Severe defects in the neonate may present with heart failure and collapse. In the older child sudden unexpected syncope and chest pain on exertion may occur.
Surgical or balloon dilatation is indicated if symptomatic, or if a high rest-ing pressure gradient of >64mmHg is present; avoidance of competitive sports recommended if severe.
Good in the majority with mild or moderate stenosis. In severe stenosis sudden death may occur. Eventually, aortic valve replacement will be re-quired.
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