Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study
Open accessAbstract
BACKGROUND: To study segmental structural and functional aortic properties in Turner syndrome (TS) patients. Aortic abnormalities contribute to increased morbidity and mortality of women with Turner syndrome. Cardiovascular magnetic resonance (CMR) allows segmental study of aortic elastic properties. METHOD: We performed Pulse Wave Velocity (PWV) and distensibility measurements using CMR of the thoracic and abdominal aorta in 55 TS-patients, aged 13-59y, and in a control population (n = 38;12-58y). We investigated the contribution of TS on aortic stiffness in our entire cohort, in bicuspid (BAV) versus tricuspid (TAV) aortic valve-morphology subgroups, and in the younger and older subgroups. RESULTS: Differences in aortic properties were only seen at the most proximal aortic level. BAV Turner patients had significantly higher PWV, compared to TAV Turner (p = 0.014), who in turn had significantly higher PWV compared to controls (p = 0.010). BAV Turner patients had significantly larger ascending aortic (AA) luminal area and lower AA distensibility compared to both controls (all p < 0.01) and TAV Turner patients. TAV Turner had similar AA luminal areas and AA distensibility compared to Controls. Functional changes are present in younger and older Turner subjects, whereas ascending aortic dilation is prominent in older Turner patients. Clinically relevant dilatation (TAV and BAV) was associated with reduced distensibility. CONCLUSION: Aortic stiffening and dilation in TS affects the proximal aorta, and is more pronounced, although not exclusively, in BAV TS patients. Functional abnormalities are present at an early age, suggesting an aortic wall disease inherent to the TS. Whether this increased stiffness at young age can predict later dilatation needs to be studied longitudinally.
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Proximal aortic stiffness is frequently increased in TS, even if the aorta is not dilated and the aortic valve is tricuspid.
“Proximal aortic stiffness is frequently increased in TS, even if the aorta is not dilated and the aortic valve is tricuspid.462-465”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [464]
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- Authors
- Devos, D. G. H., De Groote, K., Babin, D., Demulier, L., Taeymans, Y., Westenberg, J. J., Van Bortel, L., Segers, P., Achten, E., De Schepper, J., & Rietzschel, E.
- Affiliations
- Ghent University Hospital (BE)
- Ghent University (BE)
- Leiden University Medical Center (NL)
- Corresponding author
- Devos, Daniel G.H.
- ORCID
- Venue
- Journal of Cardiovascular Magnetic Resonance
- Publisher
- Elsevier BV
- Published
- 2016-12
- Volume
- 19
- Issue
- 1
- Pages
- 27
- ISSN
- 1097-6647 (ISSN-L)
- PMC
- PMC5320803
- OpenAlex
- W2589519033
- Cited by
- 29
- References
- 52
- Citations by year
- 2025: 2 · 2024: 3 · 2023: 2 · 2022: 4 · 2021: 3 · 2020: 3 · 2019: 5 · 2018: 6 · 2017: 1
- FWCI
- 3.29
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Journal of Cardiovascular Magnetic Resonance (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion, cc-by)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Leiden Repository (Leiden University) (submittedVersion)
- VUBIR (Vrije Universiteit Brussel) (submittedVersion)
- Funders
- IWT innovation (130865)
- KOF clinical fellowship of the Ghent University Hospital (AB/SDS/799/2008/hdp)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Health and Disease Prevention
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Keywords
- Turner syndrome, aortic stiffness, aortic dilatation, pulse wave velocity, aortic distensibility, bicuspid aortic valve, tricuspid aortic valve, ascending aorta, aortic wall disease, cardiovascular magnetic resonance
- MeSH terms
- Bicuspid Aortic Valve Disease, Adolescent, Adult, Aorta, Abdominal, Aorta, Thoracic, Aortic Diseases, Aortic Valve, Child, Dilatation, Pathologic, Female, Heart Valve Diseases, Humans, Image Interpretation, Computer-Assisted, Middle Aged, Predictive Value of Tests, Prospective Studies, Risk Factors, Turner Syndrome, Case-Control Studies, Magnetic Resonance Imaging, Cine, Young Adult, Vascular Stiffness, Pulse Wave Analysis
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