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Purpose : Bicuspid aortic valve (BAV) is the most common congenital heart defect. Children with BAV are more likely to have valve dysfunction and to require intervention during childhood. According to the subtype of BAV, prognosis and treatment may be different. The aim of this study was to assess feasibility,reproductibility and accuracy of 3D echocardiography (3DE) in order to diagnose BAV and to depict accurately the leaflets morphology.Methods: 72 consecutive children with suspicion of BAV were included in a prospective monocentric study. 2DE and 3DE views were recorded by the same investigator. Images were analysed separately by two confirmed pediatric cardiologists in order to assess BAV. Inter and intra-observer concordance were evaluated in the subgroup of the last 18 patients. We compared 2DE and 3DE for the description of the spatial position of cusps and raphes. The association with aortic aneurysms and coarctation, aortic valve insufficiency or stenosis were reported.Results: Median age was 5 years[IQR 1,4 – 11,3]. 3DE feasibility was 100%. Inter and intraobserver concordance were excellent with both a κ of 0,93. Mean time acquisition was 2,3 minutes. Using 3DE, BAV was not found in 11,1%[CI 95%, 5,0-20,7] of suspected patients on 2DE. Only 44,4% of uncertain BAV on 2DE were confirmed by 3DE. For 34,4% of patients [CI 95%, 22,9-47,3], 3DE allowed a better visualization of the leaflets morphology leading to a reclassification of the BAV. There was a moderate correlation (κ=0,57) between 2DE and 3DE for the classification of BAV according to the raphe localization.Conclusions: 3DE is a simple, rapid and reliable method for the diagnosis and the accurate description of BAV in children. This technique may be particularly helpful in order to precise the prognosis or to guide the surgeon.