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Published on: May 16, 2025
Hypertension is a major risk factor for aortic root dilatation in women with Turner's syndrome
M Elsheikh1, B Casadei, G S Conway
1Department of Endocrinology, Radcliffe Infirmary, Woodstock Road, Oxford, OX2 6HE, UK.
Insights
Women with Turner's syndrome (TS) face higher mortality from cardiovascular issues. This study found aortic root dilatation in 42% of TS patients, linked to hypertension and bicuspid aortic valves, necessitating regular screening.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Turner's syndrome (TS) is associated with a threefold increase in mortality, predominantly due to cardiovascular complications.
- Aortic dissection represents a significant and potentially fatal cause of mortality in women with TS.
Purpose of the Study:
- To determine the prevalence of aortic root dilatation in women with Turner's syndrome.
- To identify factors contributing to the development of aortic root dilatation in this population.
Main Methods:
- Clinical examination and echocardiography (M-mode and 2D) were performed on 38 women with TS.
- Aortic root dilatation was defined as a diameter exceeding the 95th percentile for body surface area.
- Serum lipid profiles and central arterial stiffness were assessed; hypertension and bicuspid aortic valves were noted.
Main Results:
- 42% of women with TS exhibited ascending aortic root dilatation.
- Aortic root dilatation was significantly associated with systolic blood pressure and left ventricular thickness.
- Hypertension was present in 50% of subjects, and 33% had a bicuspid aortic valve.
Conclusions:
- Aortic root dilatation is a prevalent and significant risk in women with Turner's syndrome, strongly correlated with blood pressure.
- The findings suggest a mesenchymal defect rather than atherosclerosis contributes to aortic dilatation.
- Regular monitoring of aortic root diameter and aggressive management of hypertension are crucial for preventing aortic dissection in TS patients.
Abstract:
Women with Turner's syndrome (TS) have a threefold increase in mortality, primarily as a result of their cardiovascular complications. Recently, the risk of fatal aortic dissection has come to light as a major cause of mortality in women with TS. The aim of this study was to assess the prevalence of aortic root dilatation in a group of women with TS and to investigate the factors contributing to its development. Thirty-eight women with TS attending a dedicated adult Turner clinic were examined clinically and by M-mode and two-dimensional echocardiography on at least one occasion. Aortic root dilatation was defined as an aortic root diameter greater than the 95th centile for body surface area. Fasting serum lipid concentrations were measured in all women. Additionally, 18 subjects underwent noninvasive assessment of central arterial stiffness using applanation tonometry. Fifty percent of subjects were hypertensive and a similar number had an abnormal echocardiogram. A bicuspid aortic valve was present in 33% of subjects, 16 women (42%) had ascending aortic root dilatation. This was associated with a bicuspid aortic valve in four women and hypertension in 11. Two women had isolated aortic root dilatation. Aortic root diameter was significantly associated with systolic blood pressure (r = 0.5, P = 0.003) and left ventricular thickness (r = 0.5, P = 0.02). There was no association with serum lipids or arterial compliance. Structural cardiac abnormalities are present in up to 50% of women with Turner's syndrome. Aortic root dilatation is a significant risk in women with Turner's syndrome and is closely dependent on blood pressure. Aortic root dilatation does not appear to be related to atherosclerosis and is more likely to be due to a mesenchymal defect. Regular surveillance of the aortic root diameter is essential in all women with Turner's syndrome and hypertension should be treated aggressively when present in order to minimize the risk of potentially fatal aortic dissection.
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