The 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, the prognosis and the treatment may be different.

The aim of this study was to assess the accuracy of 3D echocardiography (3DE) in order to diagnose BAV and to depict accurately the leaflets morphology.

40 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 reviewed separately by two confirmed pediatric cardiologists in order to assess BAV. Then, we compared 2DE and 3DE for the description of the spatial position of cusps and raphes. The association with aortic aneurysms, aortic valve insufficiency or stenosis and other cardiac malformation were also reported.

The median age was 4.8 years (±7.3). For 19 patients (47%), 3DE allows a better visualization of the leaflets morphology leading to a reclassification of the BAV. Raphes are better distinguished by 3D live (sensitivity=100% [CI95%, 88–100], specificity=100% [CI95%, 67–100]) or biplane echocardiography (sensitivity=96% [CI95%, 80–99], specificity=63% [CI95%, 35–85]) than 2DE (sensitivity=86% [CI95%, 62–96], specificity=28% [14–50]). Inter-observer variability is almost null.

3D live and biplane echocardiography 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.