Summary: Bicuspid aortic valve (BAV) is an independentrisk factor for aneurysm and dissection of the ascending aorta. Despite this association, routine imaging of the aorta has not been recommended for patients with BAV. We describe two young men who developed life-threatening aneurysm or dissection ofthe ascending aorta; one had a normally functioning BAV and the other was 10 years after valve replacement. The pathology ofthis condition is very similar to that found in the Marfan syndrome. We recommend echocardiographic surveillance ofthe ascending aorta at regular intervals, and considcration of beta-adrenergic blockade among patients with significant dilation.

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