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Cited by 60Aortic Disease and Treatment ApproachesConnective tissue disorders researchGenetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities

Aortic dilatation and outcome in women with Turner syndrome

Duijnhouwer, Anthonie L, Bons, Lidia R, Timmers, Henri J L M, van Kimmenade, Roland R L, Snoeren, Miranda, Timmermans, Janneke, van den Hoven, Allard T, Kempers, Marlies +3 more·2019·Heart
DOI 10.1136/heartjnl-2018-313716
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Abstract

Background Women with Turner syndrome (TS) are at increased risk of aortic dissection, which is related to ascending aortic diameter. However, the relation between aortic diameter and outcome is not well determined. This study evaluates the prevalence of aortic dilatation, the growth rate of the aorta and the risk of aortic complications in adults with TS. Methods Single centre, retrospective study of all women with TS followed with a strict protocol in an outpatient TS clinic. Aortic diameters were analysed using advanced imaging. The primary outcome was a combined endpoint of aortic-related mortality, aortic dissection and preventive aortic surgery. The secondary endpoint was aortic growth and prevalence of aortic dilatation, defined as an aortic size index >20 mm/m 2 at baseline. Results At least one cardiac MR/CT was available in 268 women with TS, having median age of 28.7 (IQR: 21.3–39.7) years. Aortic dilatation was present in 22%. Linear regression identified independent factors associated with larger aortic diameters: age (coefficient=0.23; p<0.001), hypertension (coefficient=2.7; p<0.001), bicuspid aortic valve (coefficient=3.3; p<0.001), 45XO karyotype (coefficient=1.7; p=0.002), weight (coefficient=0.075; p<0.001) and growth hormone treatment (coefficient=1.4; p=0.044). During follow-up (6.8±3.2 years), five women (2%) reached the primary endpoint (two dissections, three aortic surgery). Women withmore than one scan (n=171; 1015 patient-years follow-up), the median aortic growth was 0.20 (IQR: 0.00–0.44) mm/year. In multivariate analysis, aortic growth was not associated with baseline aortic diameter or other variables. Conclusions Aortic dilatation is common and known associations were confirmed in large adult TS cohort However, aortic dissection, related mortality and preventive aortic surgery are rare. Growth hormone treatment in childhood was associated with aortic dimensions.

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Crossref · 10.1136/heartjnl-2018-313716

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  • Non-invasive imaging is an essential part of cardiovascular screening and surveillance across the life span of individuals with TS.

    “Non-invasive imaging is an essential part of cardiovascular screening and surveillance across the life span of individuals with TS,233,388,396,418-423 but imaging remains underutilized and not systematically applied.420,424”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [233]

  • In one study, AHI showed greater predictive value compared to the absolute diameter, Z-score, or ASI.

    “In practice, AHI is easiest to use and in one study showed greater predictive value compared to the absolute diameter, Z-score, or ASI. This is probably due to the prevalence of obesity, which can deflate the ASI or Z-score (Table 13).233,234,445”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [233]

  • There is currently insufficient evidence to recommend one indexing method over another.

    “There is currently insufficient evidence to recommend one indexing method over another.233”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [233]

  • The most frequently used indexing methods are the Z-score, the aortic height index (AHI), and the aortic size index (ASI).

    “The most frequently used indexing methods are the Z-score (Z, dimensionless unit indicating the number of standard deviations from the population mean), the aortic height index (AHI, aortic diameter in millimeters divided by body length in meters), and the aortic size index (ASI, aortic diameter in centimeters divided by body surface area (BSA) per square meters) (Table 12).232,233,441-443”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [233]

  • The greater predictive value of AHI is probably due to the prevalence of obesity, which can deflate the ASI or Z-score.

    “In practice, AHI is easiest to use and in one study showed greater predictive value compared to the absolute diameter, Z-score, or ASI. This is probably due to the prevalence of obesity, which can deflate the ASI or Z-score (Table 13).233,234,445”

    From Clinical practice guidelines for the care of girls and women with Turner syndrome. (2024) · cites this paper as [233]

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Authors
Duijnhouwer, A. L., Bons, L. R., Timmers, H. J. L. M., van Kimmenade, R. R. L., Snoeren, M., Timmermans, J., van den Hoven, A. T., Kempers, M., van Dijk, A. P. J., Fleischer, K., & Roos-Hesselink, J. W.
Affiliations
  • Radboud University Medical Center (NL)
  • Erasmus MC (NL)
Corresponding author
Duijnhouwer, Anthonie L
ORCID
Venue
Heart
Publisher
BMJ
Published
2018-10-27
Volume
105
Issue
9
Pages
693-700
ISSN
  • 1355-6037 (ISSN-L)
  • 1468-201X
DOI
10.1136/heartjnl-2018-313716
OpenAlex
W2898249467
Cited by
60
References
24
Citations by year
2026: 4 · 2025: 6 · 2024: 7 · 2023: 4 · 2022: 8 · 2021: 10 · 2020: 14 · 2019: 7
FWCI
5.84
Type
journal article
Language
en
Open access
closed
Locations
  • Heart (publishedVersion)
  • PubMed (publishedVersion)
  • EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
  • HighWire Press Open Archive (submittedVersion)
  • Radboud Repository (Radboud University) (submittedVersion)
Funders
Hartstichting
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 dissection, aortic diameter, aortic growth rate, aortic-related mortality, growth hormone therapy, bicuspid aortic valve, hypertension, women
MeSH terms
Adult, Aortic Dissection, Aorta, Thoracic, Echocardiography, Electrocardiography, Female, Follow-Up Studies, Humans, Netherlands, Retrospective Studies, Tomography, X-Ray Computed, Turner Syndrome, Prevalence, Survival Rate, Aortic Aneurysm, Thoracic, Magnetic Resonance Imaging, Cine, Young Adult

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