Aortic dimensions in Turner syndrome

Abstract

In Turner syndrome, linear growth is less than the general population. Consequently, to assess stature in Turner syndrome, condition-specific comparators have been employed. Similar reference curves for cardiac structures in Turner syndrome are currently unavailable. Accurate assessment of the aorta is particularly critical in Turner syndrome because aortic dissection and rupture occur more frequently than in the general population. Furthermore, comparisons to references calculated from the taller general population with the shorter Turner syndrome population can lead to over-estimation of aortic size causing stigmatization, medicalization, and potentially over-treatment. We used echocardiography to measure aortic diameters at eight levels of the thoracic aorta in 481 healthy girls and women with Turner syndrome who ranged in age from two to seventy years. Univariate and multivariate linear regression analyses were performed to assess the influence of karyotype, age, body mass index, bicuspid aortic valve, blood pressure, history of renal disease, thyroid disease, or growth hormone therapy. Because only bicuspid aortic valve was found to independently affect aortic size, subjects with bicuspid aortic valve were excluded from the analysis. Regression equations for aortic diameters were calculated and Z-scores corresponding to 1, 2, and 3 standard deviations from the mean were plotted against body surface area. The information presented here will allow clinicians and other caregivers to calculate aortic Z-scores using a Turner-based reference population. © 2015 Wiley Periodicals, Inc.

Aortic dilatation and outcome in women with Turner syndrome

“…As shown by others, current study shows that also in women with TS age has a clear impact on aortic diameter 15. This association with age is also observed in the general population 13 16.…”

Allometric considerations when assessing aortic aneurysms in Turner syndrome: Implications for activity recommendations and medical decision‐making

“…Accordingly, Quezada et al recently reported Turner syndrome-specific aortic Z-score formulae by using healthy girls and women with Turner syndrome as the reference population. [Quezada et al, 2015] In that study, the resulting aortic Z-scores estimates were predicted to be lower compared to Z-scores calculated using a general population reference. However, the relationship between Z-scores derived from children with Turner syndrome versus a general population (hereafter referred to as “pediatric/young adult based Z-scores”) has not been previously reported.…”

“…[Quezada et al, 2015] Briefly, 458 subjects with Turner syndrome ages 2 to 65 had focused echocardiograms performed, as part of the Turner syndrome Healthy Heart Project, and all were performed at the annual meetings of the Turner syndrome Society of the United States. The Oregon Health & Science Institutional Review Board approved the protocol and continues to oversee this ongoing longitudinal study.…”

“…The focused echo protocol used in this study has been previously described and was performed in accordance with the standard guidelines. [Quezada et al, 2015] [Lopez et al, 2010]…”

“…All data utilized for the current analysis has been reported previously (Quezada et al, 2015). Briefly, 458 subjects with Turner syndrome ages 2-65 had focused echocardiograms performed as part of the Subjects included in the study were healthy females with Turner syndrome.…”