TY  - EJOU
AU  - Niaz, Talha 
AU  - Poterucha, Joseph T. 
AU  - Johnson, Jonathan N. 
AU  - Craviari, Cecilia 
AU  - Nienaber, Thomas 
AU  - Palfreeman, Jared 
AU  - Cetta, Frank 
AU  - Hagler, Donald J. 

TI  - Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects
T2  - Structural and Congenital Heart Disease

PY  - 2017
VL  - 12
IS  - 3
SN  - 3071-1738

AB  - <b>Background:</b> 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.<br/>
<b>Methods:</b> 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.<br/>
<b>Results:</b> 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).<br/>
<b>Conclusions:</b> 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.
KW  - ascending aorta dilation
KW  -  aortic valve stenosis
KW  -  bicuspid aortic valve
KW  -  congenital heart defects
KW  -  sinus of Valsalva

DO  - 10.1111/chd.12429