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 diameter (AoRd)/yr was 0.22 cm vs. 0.08 cm. Some statistically significant clinical and echocardiographic predictors of rate of AoR dilation were found, but appeared to explain little of the overall variation in AoRz and AoRd (see Table). Generalized dilatation of the aortic root, sinotubular junction and ascending aortic dimensions predicted referral for aortic surgery (see Table). A yearly change in AoRz/yr of 0.72 (sensitivity 42%, specificity 92%) and in AoRd/yr of 0.34 cm (sensitivity 38%, specificity 95%) predicted referral for aortic surgery.
Conclusions: In this cohort of young MFS, no robust predictors of more rapid AoR dilation were identified; however, age, gender, race and increased sinotubular junction appear contributory. Generalized proximal aortic dilatation predicted timing of referral for aortic surgery. Rate of AoR dilation cut-points had high specificity, but low sensitivity for predicting referral for aortic surgery, limiting their clinical use.
Author Disclosures: A. Hoskoppal: None. S. Menon: None. F. Trachtenberg: None. J.C. Levine: None. E. Selamet Tierney: None. K.M. Burns: None. J. De Backer: None. B.D. Gelb: None. M. Gleason: None. J. James: None. W.W. Lai: None. A. Liou: None. L. Mahony: None. A.K. Olson: None. R.E. Pyeritz: None. A.M. Sharkey: None. M. Stylianou: None. S.B. Wechsler: None. L. Young: None. R.V. Lacro: None. T.J. Bradley: None.
- © 2016 by American Heart Association, Inc.