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

Pediatric Heart Network Investigators

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1094-1101
Number of pages8
JournalAmerican Journal of Cardiology
Issue number9
StatePublished - May 1 2018

Bibliographical note

Funding Information:
This study was supported by U01 grants from the National Heart, Lung, and Blood Institute ( HL068269 , HL068270 , HL068279 , HL068281 , HL068285 , HL068292 , HL068290 , HL068288 , HL085057 ) and the Food and Drug Administration Office of Orphan Products Development . Additional support was provided by the Marfan Foundation , Merck & Co., Inc. , and Teva Canada Limited .

Publisher Copyright:
© 2018 Elsevier Inc.


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