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Shape of the dilated aorta in children with bicuspid aortic valve
Christopher R Mart1, Bryn E McNerny2
1Department of Pediatrics, University of Utah/Primary Children's Medical Center, Salt Lake City, Utah, USA.
Insights
The shape of the dilated aorta in children with bicuspid aortic valve varies, with multiple patterns observed. As dilation increases, a single shape (S3) becomes dominant, particularly after age six.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Congenital Heart Disease
Background:
- Bicuspid aortic valve (BAV) is associated with aortic dilation in adults.
- Aortic shape variations in pediatric BAV patients are not well-characterized.
- Understanding aortic morphology in pediatric BAV is crucial for risk stratification.
Purpose of the Study:
- To investigate the diversity of aortic shapes in children with BAV.
- To determine associations between aortic shape and patient demographics, hemodynamics, and dilation severity.
- To identify patterns in aortic dilation among pediatric BAV patients.
Main Methods:
- Retrospective review of 187 echocardiograms in pediatric patients (0-18 years) with BAV.
- Assessment of aortic valve morphology, aortic shape/size, and hemodynamic parameters.
- Quantification of aortic dilation using z-scores and classification of aortic shapes (S1-S6).
Main Results:
- Aortic dilation was identified in 104 of 187 patients.
- Six distinct aortic shapes (S1-S6) were observed.
- Shape S3 was most frequent in children over six and correlated with greater global aortic enlargement; no significant associations with gender, valve morphology, or hemodynamics were found.
Conclusions:
- Aortic dilation in pediatric BAV patients exhibits diverse shapes, not a uniform pattern.
- Shapes S2 and S3 are the most prevalent.
- Progressive aortic dilation leads to a predominant S3 shape.
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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