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


Journal

European heart journal. Cardiovascular Imaging
ISSN: 2047-2412
Titre abrégé: Eur Heart J Cardiovasc Imaging
Pays: England
ID NLM: 101573788

Informations de publication

Date de publication:
01 Oct 2019
Historique:
received: 17 12 2018
accepted: 15 03 2019
pubmed: 23 7 2019
medline: 1 12 2020
entrez: 23 7 2019
Statut: ppublish

Résumé

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. 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). Aortic growth rates in TS are not increased. BAVs and CoA are major factors that impact aortic growth. Aortic outcomes remain a concern.

Sections du résumé

BACKGROUND 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 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 CONCLUSIONS
Aortic growth rates in TS are not increased. BAVs and CoA are major factors that impact aortic growth. Aortic outcomes remain a concern.

Identifiants

pubmed: 31329837
pii: 5435910
doi: 10.1093/ehjci/jez065
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1164-1170

Informations de copyright

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

Auteurs

Kristian H Mortensen (KH)

Department of Endocrinology and Internal Medicine, and Medical Research Laboratories, Aarhus University Hospital, Aarhus N, Denmark.
Cardiorespiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Jan Wen (J)

Department of Endocrinology and Internal Medicine, and Medical Research Laboratories, Aarhus University Hospital, Aarhus N, Denmark.

Mogens Erlandsen (M)

Section for Biostatistics, Department of Public Health, Aarhus University, Aarhus C, Denmark.

Christian Trolle (C)

Department of Endocrinology and Internal Medicine, and Medical Research Laboratories, Aarhus University Hospital, Aarhus N, Denmark.

Steffen Ringgaard (S)

MR Research Centre, Aarhus University Hospital, Aarhus N, Denmark.

Ephraim J Gutmark (EJ)

Department of Aerospace Engineering and Engineering Mechanics, CEAS, University of Cincinnati, Cincinnati, OH, USA.

Iris Gutmark-Little (I)

Division of Endocrinology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Niels H Andersen (NH)

Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark.

Claus H Gravholt (CH)

Department of Endocrinology and Internal Medicine, and Medical Research Laboratories, Aarhus University Hospital, Aarhus N, Denmark.
Department of Molecular Medicine, Aarhus University Hospital, Aarhus N, Denmark.

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