Cited by 41Cardiovascular Health and Disease PreventionAortic Disease and Treatment ApproachesConnective tissue disorders research

Increased aortic stiffness in prepubertal girls with Turner syndrome

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De Groote, Katya, Devos, Daniel, Van Herck, Koen, De Wolf, Daniel, Van der Straaten, Saskia, Rietzschel, Ernst, Raes, Ann, Vandekerckhove, Kristof +3 more·2017·Journal of Cardiology

Abstract

Background Aortic dilation and dissection contribute highly to the increased mortality of Turner syndrome (TS) but the exact pathophysiology is not completely understood.Design Prospective case - control study.Methods 15 prepubertal TS girls (median age 10.64, IQ 8.31-11.04) with a tricuspid (TAV, n=9) or a bicuspid (BAV, n=6) aortic valve, and 31 sex-, age-, and height-matched healthy controls underwent a cardiac and vascular ultrasound to evaluate aortic dimensions and elastic properties of the aortic wall.Results TS BAV had significantly larger ascending aortic diameters than controls for absolute diameter, 22.2±5.1mm vs. 18.6±1.9mm (p=0.014) and z-score 1.7±2.1 vs. 0.1±0.7 (p=0.008). Distensibility of the ascending aorta was lower in the TS than in controls (40.2×10-3 kPa-1, IQ 31.3-56.2 vs. 62.9×10-3 kPa-1, IQ 55.5-76.5, p=0.003), both for TS TAV (p=0.014) and BAV (p=0.005). Stiffness index was higher in TS than in controls (5.26, IQ 3.34-5.26 vs. 3.23, IQ 2.55-3.24, p=0.005), both for TS TAV (p=0.028) and TS BAV (p=0.006). Pulse wave velocity was not different between groups. There was no correlation between stiffness and z-score of the ascending aortic diameter.Conclusions In prepubertal TS girls, stiffness of the ascending aorta is increased in patients with a BAV and TAV while dilation of the ascending aorta is more frequent in BAV. This suggests an intrinsic aortic wall abnormality making all TS patients at increased risk for severe aortic complications although the risk is the highest for TS with BAV.

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Authors
De Groote, K., Devos, D., Van Herck, K., De Wolf, D., Van der Straaten, S., Rietzschel, E., Raes, A., Vandekerckhove, K., Panzer, J., De Wilde, H., & De Schepper, J.
Affiliations
Ghent University Hospital (BE)
Venue
Journal of Cardiology
Publisher
Elsevier BV
Published
2017-01
Volume
69
Issue
1
Pages
201-207
ISSN
0914-5087 (ISSN-L)
OpenAlex
W2321483747
Cited by
41
References
41
Citations by year
2025: 3 · 2024: 6 · 2023: 2 · 2022: 6 · 2021: 3 · 2020: 2 · 2019: 9 · 2018: 8 · 2017: 1 · 2016: 1
FWCI
5.18
Type
journal article
Language
en
Open access
Locations
  • Journal of Cardiology (publishedVersion)
  • PubMed (publishedVersion)
  • Ghent University Academic Bibliography (Ghent University) (submittedVersion)
  • VUBIR (Vrije Universiteit Brussel) (submittedVersion)
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Health and Disease Prevention
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Keywords
Turner syndrome, aortic stiffness, ascending aorta, bicuspid aortic valve, tricuspid aortic valve, aortic dilatation, aortic distensibility, prepubertal girls, aortic complications
MeSH terms
Aorta, Aortic Diseases, Aortic Valve, Child, Dilatation, Pathologic, Female, Heart Valve Diseases, Humans, Prospective Studies, Turner Syndrome, Case-Control Studies, Vascular Stiffness, Pulse Wave Analysis

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