Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?

Aortic root (AoR) size remains an imperfect predictor of rate of aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such as aortic stiffness have been proposed as additional predictors. In this study we assessed the rate of AoR dilation and stiffness in adult patients with MF...

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Bibliographic Details
Published in:JTCVS open 2022-06, Vol.10, p.113-120
Main Authors: Cox, Kelly, Oquendo, Yousi A., Liang, David, Selamet Tierney, Elif Seda
Format: Article
Language:English
Subjects:
Adult: Aorta
aortic root
dilation
Marfan syndrome
stiffness
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Summary:Aortic root (AoR) size remains an imperfect predictor of rate of aortic dilation in Marfan syndrome (MFS). Indicators of vascular phenotype such as aortic stiffness have been proposed as additional predictors. In this study we assessed the rate of AoR dilation and stiffness in adult patients with MFS. We performed a retrospective chart review. We included adult patients with MFS (aged 20-40 years) with at least 2 local echocardiograms 6 months apart (no aortic surgery in-between). A blinded observer analyzed the echocardiograms. AoR dilation rate and stiffness were calculated. Thirty-two patients (53% women; median age, 21.1; interquartile range [IQR], 19-24 years at first echocardiogram) were included. AoR dilation rate in the entire cohort was 0 to 8 mm/year (median, 0.465; IQR, 0.23-1.45 mm/year). Multiple linear regression analysis showed that baseline AoR stiffness was associated with AoR dilation rate (β = 0.0004; P 
ISSN:2666-2736
2666-2736
DOI:10.1016/j.xjon.2022.04.011