The Bicuspid Aortic Valve: An Integrated Phenotypic Classification of Leaflet Morphology and Aortic Root Shape

Benjamin M Schaefer ^1*, Mark B Lewin ², Karen K Stout ², Edward Gill ², Allison Prueitt ², Peter H Byers ² and Catherine M Otto ²

¹ Northeast Cardiology Associates, United States
² University of Washington School of Medicine, United States

^* To whom correspondence should be addressed. E-mail: benschae{at}u.washington.edu.

Accepted 8 January 2008

Abstract

OBJECTIVE To establish a classification of bicuspid aortic valve (BAV) that includes both leaflet morphology and aortic shape.

SETTING Two academic medical centers of the University of Washington, Seattle.

PATIENTS 191 adult patients with BAV.

INTERVENTIONS Review of clinical data and transthoracic echocardiograms.

MAIN OUTCOME MEASURES Assessment of leaflet morphology; valve function; aortic shape and dimensions.

RESULTS We identified three morphologies: Type 1, fusion of right and left coronary cusp (N=152); Type 2, right and non-coronary fusion (N=39); and Type 3, left and non-coronary fusion (N=1). Comparing Type 1 and 2 BAV, there were no significant differences in age, height, weight, blood pressure, or aortic valve function. Type 1 was more common in men (69 vs. 45%,). The aortic sinuses were larger in Type 1, while Type 2 had larger arch dimensions. Myxomatous mitral valves were more common in Type 2 BAV (13% vs. 2.6%, p < 0.05). Three aortic shapes were defined: normal (N), sinus effacement (E), and ascending dilation (A). Comparing Type 1 to Type 2 BAV, shape N was more common in Type 1 (60% vs 32%), and Type A was more common in Type 2 (35% vs 54%,), Type E was rare (p<0.01 across all groups).

CONCLUSION A comprehensive BAV phenotype includes aortic shape. Type 1 BAV is associated with male gender and normal aortic shape but a larger sinus diameter. Type 2 leaflet morphology is associated with ascending aorta dilation, larger arch dimensions and higher prevalence of myxomatous mitral valve disease.

Keywords: aortic valve dysfunction, bicuspid aortic valve, classification, diseases of the aorta, echocardiography

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