Hypertension is a major risk factor for aortic root dilatation in women with Turner's syndrome

INTRODUCTION Women with Turner's syndrome (TS) have a threefold increase in mortality, primarily as a result of their cardiovascular complications. Re cently, the risk of fatal aortic dissection has come to light as a major ca use 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 inv estigate the factors contributing to its development. METHODS Thirty-eight women with TS attending a dedicated adult Turner clini c were examined clinically and by M-mode and two-dimensional echocardiograp hy on at least one occasion. Aortic root dilatation was defined as an aorti c root diameter greater than the 95th centile for body surface area. Fastin g serum lipid concentrations were measured in all women. Additionally, 18 s ubjects underwent noninvasive assessment of central arterial stiffness usin g applanation tonometry. RESULTS Fifty percent of subjects were hypertensive and a similar number ha d an abnormal echocardiogram. A bicuspid aortic valve was present in 33% of subjects, 16 women (42%) had ascending aortic root dilatation. This was as sociated with a bicuspid aortic valve in four women and hypertension in 11. Two women had isolated aortic root dilatation. Aortic root diameter was si gnificantly associated with systolic blood pressure (r = 0.5, P = 0.003) an d left ventricular thickness (r = 0.5, P = 0.02). There was no association with serum lipids or arterial compliance. CONCLUSIONS Structural cardiac abnormalities are present in up to 50% of wo men with Turner's syndrome. Aortic root dilatation is a significant risk in women with Turner's syndrome and is closely dependent on blood pressure. A ortic root dilatation does not appear to be related to atherosclerosis and is more likely to be due to a mesenchymal defect. Regular surveillance of t he aortic root diameter is essential in all women with Turner's syndrome an d hypertension should be treated aggressively when present in order to mini mize the risk of potentially fatal aortic dissection.