Key result
In children with bicuspid aortic valve, aortic dilation presents in multiple shapes, most commonly an enlarged ascending aorta (S2) in children under 6 years and effacement of the sinotubular ridge (S3) in older children, with S3 associated with the most severe enlargement.
Observational (n=187)
No
In children with bicuspid aortic valve, the dilated aorta exhibits multiple shapes, with the S3 pattern becoming dominant in older children and associated with the most severe degree of aortic enlargement.
May support age- and shape-specific aortic surveillance in pediatric bicuspid aortic valve; leaves open whether S3 predicts progression or events.
BACKGROUND: The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. METHODS: One hundred and eighty-seven echocardiograms done on pediatric patients (0 - 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. RESULTS: Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. CONCLUSIONS: The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern.
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Mart et al. (2013) conducted an observational in Bicuspid aortic valve (n=187). Bicuspid aortic valve was evaluated on Shape of the dilated aorta. In children with bicuspid aortic valve, aortic dilation presents in multiple shapes, most commonly an enlarged ascending aorta (S2) in children under 6 years and effacement of the sinotubular ridge (S3) in older children, with S3 associated with the most severe enlargement.
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