Abstract 18044: Predictors of Bicuspid Aortic Valve Associated Aortopathy in Pediatric Patients
Bicuspid aortic valve (BAV) is the most common congenital heart disease and is commonly associated with significant ascending aorta dilation. Valve morphology and function have been proposed as potential risk factors; however evaluating their role is difficult, as BAV morphology and valve function are inherently related. Our objective was to determine whether BAV morphology and function are independently associated with ascending aorta dilation in pediatric patients.
We performed a multicenter, retrospective, cross-sectional study of pediatric patients with BAV that were followed after 2003. The last patient echocardiogram prior to valve intervention was analyzed for BAV morphology, presence and severity of aortic stenosis (AS) and insufficiency (AI), and aortic root and ascending aorta dimensions. The relationship of potential risk factors to ascending aorta dimensions at different ages was determined using linear regression.
Data was obtained from 1725 patients (68% male). The most common BAV morphology was right-left (R-L) coronary cusp fusion (65%) followed by right-non (R-N) fusion (34%) and left-non fusion (1%). Fourteen percent of patients had at least moderate AS and 7% had at least moderate AI. R-L fusion was associated with coarctation (55%), while R-N fusion was less associated with coarctation (19%) and more associated with valve dysfunction (24% and 12% with at least moderate AS and AI). Twenty-seven percent of patients had significant ascending aorta dilation (Z score >3). AS and AI were associated with more significant ascending aorta dilation and a faster Z-score progression over time. R-N fusion was associated with ascending aorta dilation; however, when valves with AS and AI were excluded, there were no differences related to morphology. In patients with no AS or AI, 15% had significant ascending aorta dilation.
In this large pediatric cohort of patients with BAV, we show that valve morphology is not independently associated with ascending aorta dilation. Valve dysfunction (both AS and AI) is associated with more significant dilation; however, even in valves with normal function, there is significant ascending aorta dilation. We suggest that there is an inherent arterial abnormality, possibly modified by aortic flow patterns.
Author Disclosures: M. Grattan: None. A. Prince: None. A. Franco-Cereceda: None. M. Petrovic: None. S.A. Mohamed: None. B. Loeys: None. H. Dietz: None. S. Mital: None. C. Manlhiot: None. G. Andelfinger: None. L. Mertens: None.
- © 2015 by American Heart Association, Inc.