Aortic Stiffness Predicts Clinical Outcome and Rate of Aortic Root Growth in Marfan Syndrome

Background: The NHLBI Pediatric Heart Network randomized trial of atenolol vs. losartan in Marfan Syndrome demonstrated no significant treatment difference in the rate of change in body surface area adjusted maximum aortic root diameter z score (AoRz). Objectives: To report trial results on aortic stiffness and to determine whether aortic stiffness predicts clinical outcome and change in AoRz. Methods: 608 patients (6 mo - 25 yr) who met original Ghent criteria and had AoRz u003e 3 were enrolled. Echocardiograms obtained at 0, 6, 12, 24 u0026 36 months were centrally interpreted. Aortic dimensions were measured by 2D imaging, and stiffness indices were calculated for aortic root (AoR) and ascending aorta (AA). Where appropriate, stiffness measurements were indexed to 1/sqrt(R-R interval) to adjust for heart rate. Data were analyzed by multivariable mixed effects modeling and Cox regression. Results: The rate of change over three years in heart rate-adjusted AoR stiffness index differed by treatment (p = 0.016), w...