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Submitted on March 28, 2005
Accepted on June 17, 2005
Department of Endocrinology, University College London Hospitals, London, UK; Vascular Physiology Unit, Institute of Child Health, University College London, London, UK
* To whom correspondence should be addressed. E-mail: g.conway{at}ucl.ac.uk.
Context: Women with Turner Syndrome (TS) have an increased cardiovascular mortality rate from both structural and ischemic heart disease, especially aortic dissection.
Objective: We hypothesized that TS women have a fundamental arterial wall defect which may be due to genetic factors or estrogen deficiency.
Design, Setting and Patients: TS women (n = 93) were compared with normal controls (n = 25) and women with 46,XX primary amenorrhea (PA) (n = 11) with a similar history of estrogen-deficiency. Clinical parameters, aortic root diameter [ARD] and extra-aortic arterial structure (common carotid [CD] and brachial artery diameter [BD] and carotid intima-media thickness [IMT]), arterial stiffness (pulse wave velocity [PWV], augmentation index [AIx]), and endothelial function (flow-mediated dilatation [FMD]) were assessed.
Main Outcome Measures: Arterial diameters and vascular physiology parameters.
Results: Differences in arterial structure were observed between TS, normal controls and 46,XXPA women: IMT (0.61 ± 0.07vs0.55 ± 0.06vs0.60 ± 0.05 mm respectively, P < 0.001), CD (5.71 ± 0.64vs5.27 ± 0.34vs5.22 ± 0.38 mm,P < 0.001) and BD (3.29 ± 0.44vs3.06 ± 0.36vs2.97 ± 0.30 mm,P = 0.006). ARD was greater in TS than normal control women. TS status, height, weight and IMT were independently associated with increased CD after multivariate adjustment (P < 0.05). TS status, age, diastolic blood pressure and CD remained independently associated with increased IMT after multivariate adjustment (P < 0.05). PWV and FMD were similar between the three groups.
Conclusion: Women with TS have greater IMT and conduit artery diameters than normal controls. Similarly increased IMT in TS and 46,XXPA women suggests that estrogen deficiency contributes to intimal thickening. Interventional studies are required to determine the extent to which blood pressure and estrogen-deficiency may be appropriate therapeutic targets to reduce cardiovascular risk in TS.
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