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

Kelly Cox, Yousi A. Oquendo, David Liang, Elif Seda Selamet Tierney*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: 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. Methods: 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. Results: 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 < .001 for elastic modulus), whereas baseline age and baseline AoR dimension were not. Eighteen of these 32 patients (56%) eventually had AoR surgery (Sx) and 14 did not have surgery (NSx). At baseline, Sx and NSx patients were similar in age. AoR dimension was larger (Sx, 4.27 cm; IQR, 4.05-4.49 cm vs NSx, 3.73 cm; IQR, 3.37-4.09 cm; P = .011) and AoR stiffness was higher in Sx patients (beta stiffness index: median, 23.2; IQR, 17.8-28.6 vs median, 15.6; IQR, 11.6-19.7; P = .024). AoR dilation rate was greater in Sx patients, independent of baseline AoR dimension (1.63 ± 0.41 mm/year vs 0.38 ± 0.08 mm/year; P = .01). Conclusions: Our results showed that AoR dilation rate varies among adult patients with MFS and is associated with baseline AoR stiffness, measured by echocardiography. Further studies are warranted to determine how aortic stiffness can be implemented clinically to refine management in patients with MFS.

Original languageEnglish (US)
Pages (from-to)113-120
Number of pages8
JournalJTCVS Open
Volume10
DOIs
StatePublished - Jun 2022

Keywords

  • Marfan syndrome
  • aortic root
  • dilation
  • stiffness

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pulmonary and Respiratory Medicine
  • Surgery

Fingerprint

Dive into the research topics of 'Aortic root dilation in adult patients with Marfan syndrome: Does aortic root stiffness matter?'. Together they form a unique fingerprint.

Cite this