Dataset Information

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

ABSTRACT:

Background

?Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS.

Methods

?In this cross-sectional study, 52 women with TS aged 35?±?13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI). As control group, 13 women with BAV without TS and 48 healthy patients were included.

Results

?Women with TS showed a higher AA-PWV (??=?1.08, confidence interval [CI]: 0.54-1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of elastin and diminished or absent expression of contractile proteins desmin, actin, and caldesmon, as well as the progesterone receptor.

Conclusion

?Both aortic arch stiffness measurements on MRI and histomorphological changes point toward an inherent abnormal thoracic aortic wall in women with TS.

SUBMITTER: Bons LR

PROVIDER: S-EPMC7758113 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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Publications

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

Bons Lidia R LR Van Den Hoven Allard T AT Malik Maira M Van Den Bosch Annemien E AE McGhie Jacky S JS Duijnhouwer Anthonie L AL Siebelink Hans-Marc J HJ Hirsch Alexander A Devos Daniel H DH Rietzschel Ernst E von der Thüsen Jan H JH van de Laar Ingrid M B H IMBH Verhagen Judith M A JMA van der Pluijm Ingrid I Budde Ricardo P J RPJ Roos-Hesselink Jolien W JW

Aorta (Stamford, Conn.) 20201001 5

<h4>Background</h4> Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS.<h4>Methods</h4> In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ...[more]

PMID: 33368097

Similar Datasets

Project description:BackgroundSeverity of thoracic aortic disease in Turner syndrome (TS) patients is currently described through measures of aorta size and geometry at discrete locations. The objective of this study is to develop an improved measurement tool that quantifies changes in size and geometry over time, continuously along the length of the thoracic aorta.MethodsCardiovascular magnetic resonance (CMR) scans for 15 TS patients [41 ± 9 years (mean age ± standard deviation (SD))] were acquired over a 10-year period and compared with ten healthy gender and age-matched controls. Three-dimensional aortic geometries were reconstructed, smoothed and clipped, which was followed by identification of centerlines and planes normal to the centerlines. Geometric variables, including maximum diameter and cross-sectional area, were evaluated continuously along the thoracic aorta. Distance maps were computed for TS and compared to the corresponding maps for controls, to highlight any asymmetry and dimensional differences between diseased and normal aortae. Furthermore, a registration scheme was proposed to estimate localized changes in aorta geometry between visits. The estimated maximum diameter from the continuous method was then compared with corresponding manual measurements at 7 discrete locations for each visit and for changes between visits.ResultsManual measures at the seven positions and the corresponding continuous measurements of maximum diameter for all visits considered, correlated highly (R-value = 0.77, P < 0.01). There was good agreement between manual and continuous measurement methods for visit-to-visit changes in maximum diameter. The continuous method was less sensitive to inter-user variability [0.2 ± 2.3 mm (mean difference in diameters ± SD)] and choice of smoothing software [0.3 ± 1.3 mm]. Aortic diameters were larger in TS than controls in the ascending [TS: 13.4 ± 2.1 mm (mean distance ± SD), Controls: 12.6 ± 1 mm] and descending [TS: 10.2 ± 1.3 mm (mean distance ± SD), Controls: 9.5 ± 0.9 mm] thoracic aorta as observed from the distance maps.ConclusionsAn automated methodology is presented that enables rapid and precise three-dimensional measurement of thoracic aortic geometry, which can serve as an improved tool to define disease severity and monitor disease progression.Trial registrationClinicalTrials.gov Identifier - NCT01678274 . Registered - 08.30.2012.

Dataset Information

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

Background

Methods

Results

Conclusion

Publications

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

Similar Datasets

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