Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology

Heart and Vessels
Christiane PeesIna Michel-Behnke

Abstract

Turner syndrome (TS) is a common genetic disorder in females with high incidence of ascending aortic dilatation and even dissection occurring as early as in the second decade. Known risk factors (RF) are bicuspid aortic valves (BAV), coarctation of the aorta (CoA), and arterial hypertension. Since 10% of dissections occur in patients without RF, an intrinsic aortic wall abnormality has been postulated. This study aimed to investigate the elasticity of the ascending aorta as a surrogate marker of aortic wall texture. Forty-six pediatric patients with genetically proven TS were prospectively examined for the morphology of their aortic valve, and size and elasticity indices of the adjacent aorta. Cohorts of 46 female subjects with tricuspid aortic valves (TAV) and ten non-syndromic females with BAV were investigated as separate control groups. Comparison of healthy controls with TS patients revealed significantly deteriorated elasticity indices in those with TS. Furthermore, normalized aortic dimensions were greater in TS patients, but dilatations of the ascending aorta with z-score levels above two were restricted to those with BAV (14/46). Deteriorated elasticity indices were measured in TS patients, independent of aortic dilata...Continue Reading

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Citations

Aug 17, 2019·American Journal of Medical Genetics. Part a·Angela E LinClaus H Gravholt
Sep 20, 2020·Journal of Clinical Ultrasound : JCU·Xiang-Ting SongYi-Fei Rui
Dec 29, 2020·Aorta : Official Journal of the Aortic Institute at Yale-New Haven Hospital·Lidia R BonsJolien W Roos-Hesselink

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Methods Mentioned

BETA
dissection
hormone replacement therapy
dissections
sedation

Software Mentioned

SPSS Statistics

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