Background Bicuspid aortic valve (BAV) is associated with early aortopathy that may precede overt aortic dilatation. Functional assessment of aortic biomechanics and extracellular matrix (ECM) biomarkers may provide additional insight beyond conventional diameter-based evaluation. Methods In this prospective case–control study, 40 children with BAV and 40 healthy controls underwent echocardiographic assessment of ascending aortic elastic properties and left ventricular global longitudinal strain (GLS). ECM-related biomarkers were measured using ELISA. Results Children with BAV demonstrated significantly impaired aortic elastic properties, including lower aortic strain (14.79 ± 2.41 vs 21.03 ± 2.94) and distensibility (2.3 [2.0–3.15] vs 4.7 [4.1–5.7] ×10⁻³ mmHg⁻¹, p < 0.001) and higher aortic stiffness index despite preserved GLS (p > 0.05). Ascending aortic Z-scores were significantly higher in the BAV group. Serum MMP-2, MMP-9, and MMP-2/TIMP-1 ratio levels were elevated in children with BAV and were associated with impaired aortic elastic parameters. TGF-β1 levels did not differ between groups but demonstrated modest correlations with aortic biomechanics within the BAV cohort. Conclusion In children with BAV, impaired aortic biomechanics and ECM remodeling appear to precede before overt myocardial dysfunction. Integrated functional imaging and biomarker assessment may enhance detection of BAV-related aortopathy. Impact <UnorderedList Mark="Bullet"> <ItemContent> Functional impairment of aortic biomechanics and extracellular matrix remodeling are detectable in children with bicuspid aortic valve before overt aortic dilatation or myocardial dysfunction. </ItemContent> <ItemContent> This study integrates aortic strain–based functional imaging with circulating ECM biomarkers, providing pediatric evidence that biomechanical deterioration precedes structural aortopathy. </ItemContent> <ItemContent> Early functional aortic assessment may improve risk stratification and longitudinal surveillance strategies in pediatric patients with bicuspid aortic valve. </ItemContent> </UnorderedList>

Aortic elastic properties and circulating matrix biomarkers in children with bicuspid aortic valve: an exploratory study

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2026年6月27日

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Nida Gülderen Kalay Şentürk,
Ali Karakaş,
Hüseyin Karadağ,
Hazal Cansu Çulpan,
İlker Kemal Yücel,
Reyhan Dedeoğlu

摘要

Background

Bicuspid aortic valve (BAV) is associated with early aortopathy that may precede overt aortic dilatation. Functional assessment of aortic biomechanics and extracellular matrix (ECM) biomarkers may provide additional insight beyond conventional diameter-based evaluation.

Methods

In this prospective case–control study, 40 children with BAV and 40 healthy controls underwent echocardiographic assessment of ascending aortic elastic properties and left ventricular global longitudinal strain (GLS). ECM-related biomarkers were measured using ELISA.

Results

Children with BAV demonstrated significantly impaired aortic elastic properties, including lower aortic strain (14.79 ± 2.41 vs 21.03 ± 2.94) and distensibility (2.3 [2.0–3.15] vs 4.7 [4.1–5.7] ×10⁻³ mmHg⁻¹, p < 0.001) and higher aortic stiffness index despite preserved GLS (p > 0.05). Ascending aortic Z-scores were significantly higher in the BAV group. Serum MMP-2, MMP-9, and MMP-2/TIMP-1 ratio levels were elevated in children with BAV and were associated with impaired aortic elastic parameters. TGF-β1 levels did not differ between groups but demonstrated modest correlations with aortic biomechanics within the BAV cohort.

Conclusion

In children with BAV, impaired aortic biomechanics and ECM remodeling appear to precede before overt myocardial dysfunction. Integrated functional imaging and biomarker assessment may enhance detection of BAV-related aortopathy.

Impact

Functional impairment of aortic biomechanics and extracellular matrix remodeling are detectable in children with bicuspid aortic valve before overt aortic dilatation or myocardial dysfunction.

This study integrates aortic strain–based functional imaging with circulating ECM biomarkers, providing pediatric evidence that biomechanical deterioration precedes structural aortopathy.

Early functional aortic assessment may improve risk stratification and longitudinal surveillance strategies in pediatric patients with bicuspid aortic valve.