The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape

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 noncoronary 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.