PT - JOURNAL ARTICLE AU - Schaefer, B M AU - Lewin, M B AU - Stout, K K AU - Gill, E AU - Prueitt, A AU - Byers, P H AU - Otto, C M TI - The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape AID - 10.1136/hrt.2007.132092 DP - 2008 Dec 01 TA - Heart PG - 1634--1638 VI - 94 IP - 12 4099 - http://heart.bmj.com/content/94/12/1634.short 4100 - http://heart.bmj.com/content/94/12/1634.full SO - Heart2008 Dec 01; 94 AB - Objective: To establish a classification of bicuspid aortic valve (BAV) that includes both leaflet morphology and aortic shape.Setting: Two academic medical centres 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 dilatation (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 dilatation , larger arch dimensions and higher prevalence of myxomatous mitral valve disease.