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Randomized Controlled Trial
. 2018 May 1;121(9):1094-1101.
doi: 10.1016/j.amjcard.2018.01.016. Epub 2018 Feb 13.

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome

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Free PMC article
Randomized Controlled Trial

Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome

Elif Seda Selamet Tierney et al. Am J Cardiol. .
Free PMC article

Abstract

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy.

Conflict of interest statement

DISCLOSURES

None. The authors do not have any conflict of interest. The views expressed in this article are those of the authors and do not represent the official views of the National Heart, Lung, and Blood Institute (NHLBI) or the National Institutes of Health.

Figures

FIGURE 1
FIGURE 1. Aortic Measurements in Systole and Diastole
Aortic dimensions were measured by 2D imaging in systole and diastole at the aortic-valve annulus (1), aortic root (2), sinotubular junction (3), and ascending aorta (4). AO: aorta, LA: left atrium, LV: left ventricle; RPA: right pulmonary artery.
FIGURE 2
FIGURE 2. Estimated Change in Heart Rate-Corrected Aortic-Root Stiffness, by Assigned Treatment
Estimated change in heart rate-corrected aortic-root stiffness index with pointwise 95% confidence bands, by assigned treatment: Atenolol in blue, Losartan in red. sqrt = square root.
FIGURE 3
FIGURE 3. Estimated Change in Maximum Aortic-Root Z-Score (ARz) By Baseline Aortic-Root Elastic Modulus and Stiffness Index
a. Estimated Change in ARz by Baseline Aortic-Root Elastic Modulus Estimated change in maximum aortic-root z-score (ARz) with pointwise 95% confidence bands, for at or below median (blue) vs. above median (red) baseline aortic-root elastic modulus groups. AR = aortic root. b. Estimated Change in ARz by Baseline Heart Rate-Corrected Aortic-Root Stiffness Index. Estimated change in maximum aortic-root z-score (ARz) with pointwise 95% confidence bands, for at or below median (blue) vs. above median (red) baseline heart rate-corrected aortic-root stiffness index groups. AR = aortic-root; sqrt = square root
FIGURE 3
FIGURE 3. Estimated Change in Maximum Aortic-Root Z-Score (ARz) By Baseline Aortic-Root Elastic Modulus and Stiffness Index
a. Estimated Change in ARz by Baseline Aortic-Root Elastic Modulus Estimated change in maximum aortic-root z-score (ARz) with pointwise 95% confidence bands, for at or below median (blue) vs. above median (red) baseline aortic-root elastic modulus groups. AR = aortic root. b. Estimated Change in ARz by Baseline Heart Rate-Corrected Aortic-Root Stiffness Index. Estimated change in maximum aortic-root z-score (ARz) with pointwise 95% confidence bands, for at or below median (blue) vs. above median (red) baseline heart rate-corrected aortic-root stiffness index groups. AR = aortic-root; sqrt = square root

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