Dilation of the ascending aorta in Turner syndrome—a prospective cardiovascular magnetic resonance study
Open accessAbstract
BACKGROUND: The risk of aortic dissection is 100-fold increased in Turner syndrome (TS). Unfortunately, risk stratification is inadequate due to a lack of insight into the natural course of the syndrome-associated aortopathy. Therefore, this study aimed to prospectively assess aortic dimensions in TS. METHODS: Eighty adult TS patients were examined twice with a mean follow-up of 2.4 ± 0.4 years, and 67 healthy age and gender-matched controls were examined once. Aortic dimensions were measured at nine predefined positions using 3D, non-contrast and free-breathing cardiovascular magnetic resonance. Transthoracic echocardiography and 24-hour ambulatory blood pressure were also performed. RESULTS: At baseline, aortic diameters (body surface area indexed) were larger at all positions in TS. Aortic dilation was more prevalent at all positions excluding the distal transverse aortic arch. Aortic diameter increased in the aortic sinus, at the sinotubular junction and in the mid-ascending aorta with growth rates of 0.1 - 0.4 mm/year. Aortic diameters at all other positions were unchanged. The bicuspid aortic valve conferred higher aortic sinus growth rates (p < 0.05). No other predictors of aortic growth were identified. CONCLUSION: A general aortopathy is present in TS with enlargement of the ascending aorta, which is accelerated in the presence of a bicuspid aortic valve.
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The proximal aorta may dilate more rapidly in individuals with TS if BAV is present.
“The proximal aorta may dilate more rapidly in individuals with TS if BAV is present,438 but there is no evidence that overall aortic dilation rates in TS are accelerated compared to matched controls without TS.421,434”
From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [438]
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- Authors
- Mortensen, K. H., Hjerrild, B. E., Stochholm, K., Andersen, N. H., Sørensen, K. E., Lundorf, E., Hørlyck, A., Pedersen, E. M., Christiansen, J. S., & Gravholt, C. H.
- Affiliations
- Aarhus University Hospital (DK)
- Corresponding author
- Mortensen, Kristian H
- ORCID
- Venue
- Journal of Cardiovascular Magnetic Resonance
- Publisher
- Elsevier BV
- Published
- 2011-01
- Volume
- 13
- Issue
- 1
- Pages
- 24
- ISSN
- 1097-6647 (ISSN-L)
- PMC
- PMC3118376
- OpenAlex
- W2101988535
- Cited by
- 99
- References
- 24
- Citations by year
- 2025: 3 · 2024: 4 · 2023: 5 · 2022: 7 · 2021: 6 · 2020: 6 · 2019: 5 · 2018: 15 · 2017: 3 · 2016: 7 · 2015: 8 · 2014: 12 · 2013: 8 · 2012: 9
- FWCI
- 8.16
- 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)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Funders
- Hjerteforeningen
- Aase og Ejnar Danielsens Fond
- Novo Nordisk
- Snedkermester Sophus Jacobsen og Hustru Astrid Jacobsens Fond
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
- Keywords
- Turner syndrome, ascending aortic dilatation, aortopathy, aortic dissection risk, aortic dimensions, aortic growth rate, bicuspid aortic valve, sinus of Valsalva, sinotubular junction, cardiovascular magnetic resonance, risk stratification
- MeSH terms
- Adolescent, Adult, Aorta, Aortic Aneurysm, Aortic Valve, Blood Pressure, Denmark, Dilatation, Pathologic, Echocardiography, Female, Heart Defects, Congenital, Heart Rate, Humans, Image Interpretation, Computer-Assisted, Magnetic Resonance Imaging, Middle Aged, Predictive Value of Tests, Prospective Studies, Risk Factors, Time Factors, Turner Syndrome, Reproducibility of Results, Observer Variation, Chi-Square Distribution, Linear Models, Case-Control Studies, Disease Progression, Risk Assessment, Blood Pressure Monitoring, Ambulatory, Imaging, Three-Dimensional, Young Adult
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