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

Abstract

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.

Diagnosis and management of aortic dissection Task Force on Aortic Dissection, European Society of Cardiology

“…Dissection involving the descending aorta below the coarctation site is reported as a complication of pregnancy [41] . Dissection also occurs in young adults, but not in children with a bicuspid aortic valve and ascending aortic dilatation [42] .…”

Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve

“…21 However, echocardiographic identification of a BAV can be obscured in severe stenosis and after cuspal fusion secondary to inflammation. A definitive diagnosis of congenital BAV can only be made by histological examination after valve excision.…”

“…Aortic dilation should be carefully monitored by echocardiography 21 and aortic root replacement recommended more aggressively for patients with BAV 24 with aortic dilation (ie, 4 to 5 cm) than for those of patients with tricuspid valve (ie, 5 to 6 cm). Combined aortic valve repair with a valve-sparing root replacement can be performed successfully in young patients with aortic regurgitation and aortic dilation.…”

Acute aortic dissection in a 10-year-old boy with bicuspid aortic valve

“…Transthoracic echocardiography demonstrates dilatation of the ascending aorta and pericardial effusion survey with healthy primary-school children, BAV presents in 0.5% of the study population (7). Patients with BAV have greater increases in aortic dimensions than those with trileaflet valves and have a higher risk of dissection (2,3,8). Although the clinical course of ascending aortic dilatation is relatively benign, follow-up for progressive dilatation is recommended (3).…”