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

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  • Kristian H Mortensen
  • Jan Wen
  • Mogens Erlandsen
  • ,
  • Christian Trolle
  • Steffen Ringgaard
  • Ephraim J Gutmark, Department of Aerospace Engineering and Engineering Mechanics, CEAS, University of Cincinnati, Cincinnati, OH, USA; UC Department of Otolaryngology - Head and Neck Surgery, Cincinnati, OH, USA.
  • ,
  • Iris Gutmark-Little, Cincinnati Center for Growth Disorders, Division of Endocrinology, Cincinnati Children's Hospital Medical Center. Cincinnati, OH, USA.;
  • ,
  • Niels H Andersen, Aalborg University
  • ,
  • Claus H Gravholt

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.

OriginalsprogEngelsk
TidsskriftEuropean Heart Journal Cardiovascular Imaging
Vol/bind20
Nummer10
Sider (fra-til)1164-1170
Antal sider7
ISSN1525-2167
DOI
StatusUdgivet - okt. 2019

Bibliografisk note

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.

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