Aortic Dimensions in Girls and Young Women with Turner Syndrome: A Magnetic Resonance Imaging Study

Abstract

This study aimed to determine the dimensions of the thoracic aorta and the predictors of aortic dimensions in girls and young women with Turner syndrome (TS). A cross-sectional study was performed at a secondary care center. The study compared 41 TS patients with 50 healthy age-matched control subjects. The mean age of the patients was 17 +/- 3.3 years. Magnetic resonance imaging was performed for all the patients. The thoracic aortic diameters of the patients were measured at nine positions. Adjustment for body surface area (BSA) was performed. The outcome for the patients was measured in terms of absolute and BSA-adjusted aortic dilation. In TS, both the absolute and the BSA-adjusted mean aortic diameters were smaller than or comparable with those of the control subjects. However, individual aortic dilation at one to four positions was found in four TS patients according to the uncorrected data and in five TS patients after BSA-adjustment. The aortic diameters correlated with height, weight, body mass index (BMI), and BSA at all positions (R = 0.34-0.60; all p < 0.04). The diameters of the aortic arch and the descending aorta correlated with a history of aortic coarctation (R = 0.35-0.52; p < 0.03). The presence of bicuspid aortic valves correlated at the descending part of the aorta (R = 0.38; p < 0.03). The mean thoracic aortic dimensions were not enlarged in girls or young TS patients. The BSA predicted aortic size at all positions. The prevalence of aortic dilation and aneurysm was lower in this population of girls and younger women with TS than in older TS populations.

Vasculopathy in the Young Turner Syndrome Population

“…This is to be interpreted as PWVf, AIx, and YEM being representative of functional changes, which may precede the structural changes of arterial thickness (cIMT). Because our patients were 10 -30 years younger than adult TS patients previously studied (1,8,27,28), and because they had not been subjected to pro- longed environmental exposures commonly associated with atherosclerosis, our data may very well indicate that these functional changes (ie, increased vascular stiffness) could represent an important early marker of vasculopathy entirely inherent to the TS phenotype. These functional changes may be occurring before any structural damage is seen with increasing chronological age.…”

“…Studies evaluating the presence of vasculopathy in the adult TS population have identified vascular changes as early as the third decade of life (1,5,8), and…”

Prediction of aortic dilation in Turner syndrome - enhancing the use of serial cardiovascular magnetic resonance

“…The entire thoracic aorta is at risk of dilation and dissection in TS [7,11]. The present study utilised a dynamic and integrative approach to both baseline CMR data and to the longest registered prospective characterisation of aortic diameter of the entire thoracic aorta in TS.…”

Aortic Aneurysm in Children and Adolescents

“…Histological evidence of cystic medial necrosis has been reported in Turner syndrome. The prevalence of aortic dilation and aneurysm is lower in the young girls and women with Turner syndrome than in older Turner syndrome population [39,40].…”