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Original research
Aortic dilation and growth in women with Turner syndrome
  1. Frederike Meccanici1,
  2. Mathilda H Schotte1,
  3. Miranda Snoeren2,
  4. Lidia R Bons1,
  5. Allard T van den Hoven1,
  6. Isabella Kardys1,
  7. Ricardo P J Budde3,
  8. Annemien E van den Bosch1,
  9. Anthonie L Duijnhouwer4,
  10. Jolien W Roos-Hesselink1
  1. 1Cardiology, Erasmus Medical Center, Rotterdam, The Netherlands
  2. 2Radiology and Nuclear Medicine, Radboudumc, Nijmegen, The Netherlands
  3. 3Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
  4. 4Cardiology, Radboudumc, Nijmegen, The Netherlands
  1. Correspondence to Dr Jolien W Roos-Hesselink, Cardiology, Erasmus Medical Center, Rotterdam 2040, The Netherlands; j.roos{at}erasmusmc.nl

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.

  • Aortic and arterial disease
  • Aortic Aneurysm
  • Congenital heart disease
  • Computed Tomography Angiography

Data availability statement

Data are available upon reasonable request.

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Footnotes

  • Contributors Study design: FM, LRB, ATvdH, ALD, JWR-H. Data collection: FM, MHS, MS, LRB, ATvdH, ALD. Data analysis: FM, IK. Data interpretation: all authors. Manuscript draft: FM, MHS. Critical revision, editing and approval of the final manuscript: all authors. Guarantor: JWR-H.

  • Funding This research was supported by the Dutch Heart Foundation (contract grant number: 2013T093).

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were involved in the design, or conduct, or reporting, or dissemination plans of this research. Refer to the Methods section for further details.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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