Key result
Coexisting congenital heart defects affect ~39% of young BAV patients, altering morphology without worsening progression.
Why the study?
Does the presence of coexisting congenital heart defects affect the morphology and progression of bicuspid aortic valve disease in pediatric and young adult patients compared to isolated BAV?
Observational (n=1,010)
No
Does the presence of coexisting congenital heart defects affect the morphology and progression of bicuspid aortic valve disease in pediatric and young adult patients compared to isolated BAV?
In pediatric and young adult patients, the morphology and progression of bicuspid aortic valve disease differ significantly when associated with coexisting congenital heart defects compared to isolated BAV, with less progression of valve dysfunction in the CHD group.
Supports CHD-specific BAV morphology assessment in pediatrics; extends prior associations but leaves progression implications open.
BACKGROUND: Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. METHODS: The Mayo Clinic echocardiography database was retrospectively analyzed to identify pediatric and young adult patients (≤22 years) who were diagnosed with BAV from 1990 to 2015. The morphology of BAV was determined from the echocardiographic studies before any intervention. RESULTS: Overall, 1010 patients with BAV were identified, 619 (61%) with isolated BAV and 391 (39%) with BAV and coexisting CHD. The incidence of BAV was highest in patients with coarctation of the aorta (36%) and interrupted aortic arch (36%). In comparison to patients with isolated BAV, patients with BAV and left-sided obstructive lesions more frequently had right-left cusp fusion (P = .0001). BAV in patients with right-sided obstructive lesions was rare, but they more frequently had right-noncoronary or left-noncoronary cusp fusion (P = .01). No significant progression of aortic stenosis or regurgitation was observed in patients with BAV and coexisting CHD; however in patients with isolated BAV the severity of aortic regurgitation increased with age. In patients with isolated BAV, the ascending aorta diameter (z-score) increased with age, peaked around 8-9 years of age, and was larger in comparison to patients with BAV and coexisting CHD. The sinus of Valsalva diameter (z-score) in patients with BAV and ventricular septal defect was larger than isolated BAV patients after 18 years (P < .04). CONCLUSIONS: The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.
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Niaz et al. (2016) conducted an observational in Bicuspid aortic valve (BAV) (n=1,010). BAV with coexisting congenital heart defects vs. Isolated BAV was evaluated on Incidence, morphology, and disease progression of BAV. Among 1010 pediatric and young adult patients with BAV, 39% had coexisting congenital heart defects, which altered BAV morphology and aortic dilatation patterns but did not worsen disease progression.
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