Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality, occurring in 1% to 2% of the general population. Adults with degenerative aortic valve (AV) disease have been shown to have an elliptical shaped AV annulus. The goal of this study was to investigate the shape of the aortic annulus in children with BAV, coarctation of the aorta (CoA) with or without BAV, and normal controls with trileaflet AVs using 3-dimensional echocardiography (3DE). We reviewed echocardiograms of children with isolated BAV (n = 40), CoA (n = 26), and controls (n = 40) that included 3DE of the AV. Eccentricity index (EI) was defined as the ratio between the smaller and larger annular dimension. ΔD was defined as the difference between the larger and smaller annular dimension. Patients with BAV had an eccentric AV annulus compared with controls (BAV EI 0.85 ± 0.05 and control EI 0.96 ± 0.03; p <0.001). Subjects with CoA also had a more eccentric annulus than controls regardless of AV morphology (CoA 0.84 ± 0.06; p <0.001). EI was not associated with somatic growth parameters or gender. Among all patients with BAV, AV dysfunction was associated with fusion of the right and noncoronary (R-N) cusps (p <0.001), but there was no association between valve dysfunction and EI. ΔD was higher in both the BAV and CoA groups compared with the control group (BAV 3.4 ± 1.9 mm, CoA 2.8 ± 1.8 mm, and control 0.6 ± 0.4 mm; p <0.001 each). Although there was no significant correlation of ΔD with age in the control group during childhood, ΔD increased with age in the BAV and CoA groups. In conclusion, children with BAV and/or CoA have an elliptical shaped AV annulus by 3DE, which is independent of age, gender, or body surface area. AV annular eccentricity may lead to inaccurate measurement of AV annular size if measured by 2DE alone. Considering AV annular eccentricity when balloon sizing the annulus before valvuloplasty may help improve interventional results in some patients.
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