Format

Send to

Choose Destination
Eur Heart J Cardiovasc Imaging. 2019 Oct 1;20(10):1164-1170. doi: 10.1093/ehjci/jez065.

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

Author information

1
Department of Endocrinology and Internal Medicine, and Medical Research Laboratories, Aarhus University Hospital, Aarhus N, Denmark.
2
Cardiorespiratory Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
3
Section for Biostatistics, Department of Public Health, Aarhus University, Aarhus C, Denmark.
4
MR Research Centre, Aarhus University Hospital, Aarhus N, Denmark.
5
Department of Aerospace Engineering and Engineering Mechanics, CEAS, University of Cincinnati, Cincinnati, OH, USA.
6
Division of Endocrinology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
7
Department of Cardiology, Aalborg University Hospital, Aalborg, Denmark.
8
Department of Molecular Medicine, Aarhus University Hospital, Aarhus N, Denmark.

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.

KEYWORDS:

Turner syndrome; aortic coarctation; aortic disease; aortic dissection; bicuspid aortic valve; cardiovascular magnetic resonance imaging

PMID:
31329837
DOI:
10.1093/ehjci/jez065

Supplemental Content

Full text links

Icon for Silverchair Information Systems
Loading ...
Support Center