Ascending aortic aneurysm and dissection in young adults with bicuspid aortic valve: implications for echocardiographic surveillance.

Bicuspid aortic valve (BAV) is an independent risk 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 of the ascending aorta; one had a normally functioning BAV and the other was 10 years after valve replacement. The pathology of this condition is very similar to that found in the Marfan syndrome. We recommend echocardiographic surveillance of the ascending aorta at regular intervals, and consideration of beta-adrenergic blockade among patients with significant dilation.