Supravalvular Aortic Stenosis – Tubular Type
A 13-year-old boy presented with frequent episodes of syncopal attacks. Physical examination showed a systolic ejection murmur (grade IV/VI) in the aortic area with radiation into the jugular notch and along the carotid vessels. An ejection click was not heard, and the blood pressure was 120/80 mmHg in the left arm and 140/80 mmHg in the right arm. The patient had regular but unequal upper extremity pulses with prominence in the right upper extremity. He was referred to us for an evaluation of the cardiac cause of the syncopal attacks. Transthoracic echocardiography revealed a relatively small left ventricular cavity and a normal systolic function as well as concentric left ventricular hypertrophy.
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