Abstract 16994: Investigating Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome

Background: Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). Objectives: To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol vs. losartan in young MFS. Methods: The study enrolled 608 subjects (6 mo - 25 yr) who met original Ghent criteria and had a body-surface area adjusted maximum aortic root diameter z score (AoRz) > 3. Data from core lab echocardiograms at 0, 12, 24 & 36 months were included in this analysis. Repeated measures linear and logistic regression was used to determine multivariable predictors of AoR dilation. ROC curves were used to determine cut-points in AoR dilation predicting referral for aortic surgery. Results: Of 24 subjects who underwent aortic surgery, median preoperative change in AoRz/yr was 0.36 vs. -0.10 in 555 subjects without surgery and in aortic root diamet...