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Cited by 23Aortic Disease and Treatment ApproachesConnective tissue disorders researchGenetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities

Aortic dilation and growth in women with Turner syndrome

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Meccanici, Frederike, Schotte, Mathilda H, Snoeren, Miranda, Bons, Lidia R, van den Hoven, Allard T, Kardys, Isabella, Budde, Ricardo P J, van den Bosch, Annemien E +2 more·2023·Heart
DOI 10.1136/heartjnl-2022-320922
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Abstract

Objective Women with Turner syndrome (TS) are at increased risk of aortic dissection, which is a life-threatening event associated with aortic dilation. Knowledge on the development of aortic dilation over time remains limited. This study aims to describe the prevalence of aortic dilation, to find associated factors and to study aortic growth in women with TS. Methods In this prospective multicentre cohort study, consecutive adult women with genetically proven TS included between 2014 and 2016 underwent ECG-triggered multiphase CT angiography at baseline and after 3 years. Aortic diameters were measured at seven levels of the thoracic aorta using double oblique reconstruction and indexed for body surface area. Ascending aortic dilation was defined as an aortic size index >20 mm/m2. Aorta-related and cardiovascular events were collected. Statistical analysis included linear and logistic regression and linear mixed effects models. Results The cohort consisted of 89 women with a median age of 34 years (IQR: 24–44). Ascending aortic dilation was found in 38.2% at baseline. At baseline, age (OR: 1.08 (95% CI 1.03 to 1.13), p<0.001), presence of bicuspid aortic valve (BAV) (OR: 7.09 (95% CI 2.22 to 25.9), p=0.002) and systolic blood pressure (OR: 1.06 (95% CI 1.02 to 1.11), p=0.004) were independently associated with ascending aortic dilation. During a median follow-up of 3.0 (2.4–3.6) years (n=77), significant aortic growth was found only at the sinotubular junction (0.20±1.92 mm, p=0.021). No aortic dissection occurred, one patient underwent aortic surgery and one woman died. Conclusions In women with TS, ascending aortic dilation is common and associated with age, BAV and systolic blood pressure. Aortic diameters were stable during a 3-year follow-up, apart from a significant yet not clinically relevant increase at the sinotubular junction, which may suggest a more benign course of progression than previously reported.

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Authors
Meccanici, F., Schotte, M. H., Snoeren, M., Bons, L. R., van den Hoven, A. T., Kardys, I., Budde, R. P. J., van den Bosch, A. E., Duijnhouwer, A. L., & Roos-Hesselink, J. W.
Affiliations
  • Erasmus MC (NL)
  • Radboud University Nijmegen (NL)
ORCID
Venue
Heart
Publisher
BMJ
Published
2022-06-15
Volume
109
Issue
2
Pages
102-110
ISSN
  • 1355-6037 (ISSN-L)
  • 1468-201X
DOI
10.1136/heartjnl-2022-320922
OpenAlex
W4282982812
Cited by
23
References
29
Citations by year
2026: 2 · 2025: 7 · 2024: 6 · 2023: 6 · 2022: 2
FWCI
3.41
Type
journal article
Language
en
Open access
Licence
other-oa
Locations
  • Heart (publishedVersion)
  • PubMed (publishedVersion)
  • EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
  • EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
  • Radboud Repository (Radboud University) (submittedVersion)
Funders
Hartstichting (2013T093)
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, aortic dilatation, aortic growth, aortic dissection, ascending aorta, bicuspid aortic valve, systolic blood pressure, sinotubular junction, aortic diameter, women
MeSH terms
Bicuspid Aortic Valve Disease, Adult, Aortic Dissection, Aortic Diseases, Aortic Valve, Dilatation, Dilatation, Pathologic, Female, Humans, Prospective Studies, Retrospective Studies, Turner Syndrome, Cohort Studies, Young Adult

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