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Decreased Aortic Elasticity in Children With Marfan Syndrome or Loeys-Dietz Syndrome

Yohei Akazawa1, Noriko Motoki, Akira Tada

  • 1Department of Pediatrics, Shinshu University School of Medicine.

Circulation Journal : Official Journal of the Japanese Circulation Society
|October 14, 2016
PubMed

Insights

Children with Marfan syndrome (MFS) or Loeys-Dietz syndrome (LDS) may have reduced aortic elasticity even before aortic root (AoR) dilation. Lower aortic distensibility and CTD type are key factors in predicting AoR dilation in pediatric patients.

Area of Science:

  • Pediatric Cardiology
  • Connective Tissue Disorders
  • Aortic Elasticity

Background:

  • Aortic elasticity is not well understood in pediatric connective tissue disorders (CTDs), including Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), particularly in cases without aortic root (AoR) dilation.
  • This study investigates aortic elasticity in pediatric MFS and LDS patients with both dilated and non-dilated AoR.

Purpose of the Study:

  • To evaluate and compare aortic elasticity parameters in pediatric patients with MFS or LDS.
  • To determine the relationship between aortic elasticity and AoR diameter in these patients.
  • To identify predictors of AoR dilatation in pediatric CTDs.

Main Methods:

  • Transthoracic echocardiography was used to assess aortic elasticity parameters (distensibility, strain, stiffness index).
  • Measurements were taken at the AoR, sinotubular junction, ascending aorta, and descending aorta.
  • Patients were categorized into dilated (AoR Z score ≥2.5) and non-dilated (AoR Z score <2.5) groups and compared to controls.

Main Results:

  • Aortic distensibility and strain were significantly lower in MFS/LDS patients compared to controls at the AoR level.
  • AoR diameter Z score correlated significantly with aortic distensibility, strain, and stiffness index in patients.
  • Multivariate analysis identified aortic distensibility and CTD type as independent predictors of AoR dilatation.

Conclusions:

  • Reduced aortic elasticity, specifically lower distensibility, may be present in pediatric MFS/LDS patients before significant AoR dilation occurs.
  • Aortic distensibility and CTD type are important parameters for estimating the risk of AoR dilatation in these children.
  • Early assessment of aortic elasticity could aid in managing pediatric patients with CTDs.
Abstract

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