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(Circulation. 2005;111:832-834.)
© 2005 American Heart Association, Inc.

Editorial

The Bicuspid Aortic Valve

Adverse Outcomes From Infancy to Old Age

Mark B. Lewin, MD; Catherine M. Otto, MD

From the Division of Cardiology, Department of Pediatrics (M.B.L.), and the Division of Cardiology, Department of Medicine (C.M.O.), University of Washington School of Medicine, Seattle.

Correspondence to Dr Catherine M. Otto, Division of Cardiology, Box 356522, University of Washington, Seattle 98195. E-mail cmotto@u.washington.edu

Key Words: Editorials • aorta • lesion • endocarditis • surgery

An extract of the first 250 words of the full text is provided, because this article has no abstract.

The population frequency of a bicuspid aortic valve is 0.9% to 1.36%,^1–3 with a 2:1 male:female ratio. It is likely that the presence of a bicuspid aortic valve has a genetic basis, with the pattern of transmission in some families suggesting an autosomal dominant pattern of inheritance.^4,5 Epidemiological data from the Baltimore-Washington Infant Study demonstrated the familial clustering of left heart obstructive lesions (including coarctation of the aorta, aortic valve stenosis, and hypoplastic left heart syndrome).⁶ More recently, the increased risk of identifying a bicuspid aortic valve in the parent or sibling of the proband with any form of left heart obstructive lesion was described.⁷ By inference, this also suggests the potential identification of a congenitally malformed aortic valve in the presence a family member with a more complex congenital heart lesion. In addition, a bicuspid aortic valve is present in >50% of patients with aortic coarctation⁸ and in 10% to 12% of women with Turner syndrome.⁹ The specific genetic locus and protein abnormality in patients with a bicuspid aortic valve have not yet been identified, however.

See p 920

The tissue abnormality in patients with a bicuspid aortic valve is not confined to the valve leaflets; these patients are at increased risk of aortic aneurysm and dissection. At the tissue level, the aorta shows cystic medial necrosis, loss of elastic fibers, increased apoptosis, and altered smooth muscle cell alignment.¹⁰ When compared with patients with a trileaflet valve, patients with a bicuspid valve have larger aortic root dimensions and an . . . [Full Text of this Article]

Related Article:

Frequency by Decades of Unicuspid, Bicuspid, and Tricuspid Aortic Valves in Adults Having Isolated Aortic Valve Replacement for Aortic Stenosis, With or Without Associated Aortic Regurgitation

William C. Roberts and Jong M. Ko
Circulation 2005 111: 920-925. [Abstract] [Full Text]

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