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Impaired aortic distensibility and elevated central blood pressure in Turner Syndrome: a cardiovascular magnetic resonance study
Authors:Jan Wen  Christian Trolle  Mette H. Viuff  Steffen Ringgaard  Esben Laugesen  Ephraim J. Gutmark  Dhananjay Radhakrishnan Subramaniam  Philippe Backeljauw  Iris Gutmark-Little  Niels H. Andersen  Kristian H. Mortensen  Claus H. Gravholt
Affiliation:1.Department of Endocrinology and Internal Medicine and Medical Research Laboratories,Aarhus University Hospital,Aarhus C,Denmark;2.Department of Clinical Medicine, MR Research Centre,Aarhus University Hospital,Aarhus,Denmark;3.Department of Aerospace Engineering and Engineering Mechanics, CEAS,University of Cincinnati,Cincinnati,USA;4.UC Department of Otolaryngology – Head and Neck Surgery,Cincinnati,USA;5.Division of Endocrinology, Cincinnati Children’s Hospital Medical Center,University of Cincinnati College of Medicine,Cincinnati,USA;6.Department of Cardiology,Aalborg University Hospital,Aalborg,Denmark;7.Cardiovascular Imaging Department, Cardio-respiratory Unit,Great Ormond Street Hospital for Children NHS Foundation Trust,London,UK;8.Department of Molecular Medicine,Aarhus University Hospital,Aarhus,Denmark
Abstract:

Background

Women with Turner Syndrome have an increased risk for aortic dissection. Arterial stiffening is a risk factor for aortic dilatation and dissection. Here we investigate if arterial stiffening can be observed in Turner Syndrome patients and is an initial step in the development of aortic dilatation and subsequent dissection.

Methods

Fifty-seven women with Turner Syndrome (48 years [29–66]) and thirty-six age- and sex-matched controls (49 years [26–68]) were included. Distensibility, blood pressure, carotid-femoral pulse wave velocity (PWV), the augmentation index (Aix) and central blood pressure were determined using cardiovascular magnetic resonance, a 24-h blood pressure measurement and applanation tonometry. Aortic distensibility was determined at three locations: ascending aorta, transverse aortic arch, and descending aorta.

Results

Mean aortic distensibility in the descending aorta was significantly lower in Turner Syndrome compared to healthy controls (P =?0.02), however, this was due to a much lower distensibility among Turner Syndrome with coarctation, while Turner Syndrome without coarctation had similar distensibility as controls. Both the mean heart rate adjusted Aix (31.4% vs. 24.4%; P?=?0.02) and central diastolic blood pressure (78.8 mmHg vs. 73.7 mmHg; P?=?0.02) were higher in Turner Syndrome compared to controls, and these indices correlated significantly with ambulatory night-time diastolic blood pressure. The presence of aortic coarctation (r = ??0.44, P =?0.005) and a higher central systolic blood pressure (r = ??0.34, P =?0.03), age and presence of diabetes were inversely correlated with aortic distensibility in TS.

Conclusion

Aortic wall function in the descending aorta is impaired in Turner Syndrome with lower distensibility among those with coarctation of the aorta, and among all Turner Syndrome higher Aix, and elevated central diastolic blood pressure when compared to sex- and age-matched controls.

Trial registration

The study was registered at ClinicalTrials.gov (#NCT01678274) on September 3, 2012.
Keywords:
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