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Cited by 17Aortic Disease and Treatment ApproachesGenetic and Clinical Aspects of Sex Determination and Chromosomal AbnormalitiesConnective tissue disorders research

Aortic growth rates are not increased in Turner syndrome—a prospective CMR study

Mortensen, Kristian H, Wen, Jan, Erlandsen, Mogens, Trolle, Christian, Ringgaard, Steffen, Gutmark, Ephraim J, Gutmark-Little, Iris, Andersen, Niels H +1 more·2019·European Heart Journal - Cardiovascular Imaging
DOI 10.1093/ehjci/jez065
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Abstract

BACKGROUND: Aortic disease is a key determinant of outcomes in Turner syndrome (TS). The present study characterized aortic growth rates and outcomes over nearly a decade in adult women with TS. METHODS AND RESULTS: Prospective observational study assessing aortic diameters twice with cardiovascular magnetic resonance imaging in women with TS [N = 91; mean follow-up 8.8 ± 3.3 (range 1.6-12.6) years] and healthy age-matched female controls [N = 37; mean follow-up 6.7 ± 0.5 (range 5.9-8.1) years]. Follow-up also included aortic outcomes and mortality, antihypertensive treatment and ambulatory blood pressure. Aortic growth rates were similar or smaller in TS, but the variation was larger. The proximal aorta in TS grew by 0.20 ± 0.26 (mid-ascending) to 0.32 ± 0.36 (sinuses) mm/year. This compared to 0.26 ± 0.14 (mid-ascending) and 0.32 ± 0.17 (sinuses) mm/year in the controls. During 799 years at risk, 7 suffered an aortic outcome (1 aortic death, 2 aortic dissections, 2 aortic interventions, 2 surgical aortic listings) with further 2 aortic valve replacements. At baseline, two women were excluded. One died during subacute aortic surgery (severe dilatation) and one had a previously undetected type A dissection. The combined aortic outcome rate was 1126 per 100 000 observation years. The aortic and all-cause mortality rates were 1 per 799 years (125 deaths per 100 000 observation years) and 9 per 799 years (1126 deaths per 100 000 observation years). Aortic growth patterns were particularly perturbed in bicuspid aortic valves (BAV) and aortic coarctation (CoA). CONCLUSION: Aortic growth rates in TS are not increased. BAVs and CoA are major factors that impact aortic growth. Aortic outcomes remain a concern.

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Crossref · 10.1093/ehjci/jez065

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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 [421]

  • There is no evidence that overall aortic dilation rates in TS are accelerated compared to matched controls without TS.

    “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 [421]

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Authors
Mortensen, K. H., Wen, J., Erlandsen, M., Trolle, C., Ringgaard, S., Gutmark, E. J., Gutmark-Little, I., Andersen, N. H., & Gravholt, C. H.
Affiliations
  • Great Ormond Street Hospital (GB)
  • Aarhus University Hospital (DK)
  • Aarhus University (DK)
  • University of Cincinnati (US)
  • Division of Endocrinology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
  • Aalborg University Hospital (DK)
Corresponding author
Andersen, Niels H
ORCID
Venue
European Heart Journal - Cardiovascular Imaging
Publisher
Oxford University Press (OUP)
Published
2019-04-09
Volume
20
Issue
10
Pages
1164-1170
ISSN
  • 2047-2404 (ISSN-L)
  • 2047-2412
DOI
10.1093/ehjci/jez065
OpenAlex
W2935852340
Cited by
17
References
24
Citations by year
2025: 3 · 2024: 2 · 2023: 3 · 2022: 4 · 2020: 3 · 2019: 2
FWCI
1.65
Type
journal article
Language
en
Open access
closed
Licence
academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model (vor, 1 d)
Locations
  • European Heart Journal - Cardiovascular Imaging (publishedVersion)
  • PubMed (publishedVersion)
  • VBN Forskningsportal (Aalborg Universitet) (submittedVersion)
Funders
  • Lundbeck
  • Danish Heart Foundation
  • Augustinus Foundation
  • Toyota Foundation
  • Aase og Einar Danielsen
  • Novo Nordisk Foundation (NNF13OC0003234, NNF15OC0016474, Hede Nielsen foundation, Aarhus University)
Topics
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Aortic Disease and Treatment Approaches
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Keywords
Turner syndrome, aortic growth rate, aortic dilatation, aortic dissection, aortic valve replacement, coarctation of the aorta, bicuspid aortic valve, cardiovascular magnetic resonance, adult women
MeSH terms
Adult, Aged, Aorta, Thoracic, Aortic Diseases, Dilatation, Pathologic, Echocardiography, Female, Humans, Middle Aged, Predictive Value of Tests, Prospective Studies, Turner Syndrome, Case-Control Studies, Disease Progression, Blood Pressure Monitoring, Ambulatory, Magnetic Resonance Imaging, Cine

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