This noncyanotic lesion can be life threatening and may present at any age. Older children may have a history of exercise intolerance with easy fatigability and chest pain. Prominent physical findings are a systolic ejection click and a diamond-shaped murmur that radiates to the neck and is associated with a suprasternal thrill. Left ventricular hypertrophy with strain can be present on ECG, and the chest x-ray may show poststenotic dilatation of the aorta, although neither of these signs is present consistently.
Syncope without cyanosis caused by critical aortic stenosis can portend a sudden life-threatening dysrhythmia. Patients should be kept strictly at rest and should be sedated, if necessary. Immediate referral for further diagnosis and possible urgent surgical repair is indicated.
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