Supravalvular aortic stenosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Most uncommon cause of left ventricular outflow tract obstruction accounting for 8% of congenital LVOT obstruction.

Anatomy

1. Obstruction occurs just above the coronary ostium at the level of the sinotubular junction:
   • Hourglass type (the most common)
   • Hypoplastic type: uniform narrowing of the ascending aorta.
2. Associated lesion is peripheral pulmonary arterial stenosis
3. Because of high perfusion pressure of the coronary arteries there is premature CAD.
4. Coronary arteries may be obstructed by an adjacent stenotic ring.

Clinical Features

1. 1/3rd of cases are transmitted as an autosomal dominant trait.
2. 50% have a characteristically greater pulse and systolic blood pressure in the right carotid and brachial arteries than in the left.
3. The systolic murmur is maximal below the right clavicle and radiates primarily to the right carotid artery.
4. No ejection click, no diastolic murmur.

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