Vasculopathy in Turner syndrome: arterial dilatation and intimal thickening without endothelial dysfunction.
BACKGROUND
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 that may be due to genetic factors or estrogen deficiency.


METHODS
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, extraaortic arterial structure [common carotid (CD), brachial artery diameter, and carotid intima-media thickness (IMT)], arterial stiffness (pulse-wave velocity, augmentation index), and endothelial function (flow-mediated dilatation) were assessed.


METHODS
These included arterial diameters and vascular physiology parameters.


RESULTS
Differences in arterial structure were observed among TS, normal controls, and 46,XX PA women: IMT (0.61 +/- 0.07 vs. 0.55 +/- 0.06 vs. 0.60 +/- 0.05 mm, respectively; P < 0.001), CD (5.71 +/- 0.64 vs. 5.27 +/- 0.34 vs. 5.22 +/- 0.38 mm; P < 0.001), and brachial artery diameter (3.29 +/- 0.44 vs. 3.06 +/- 0.36 vs. 2.97 +/- 0.30 mm; P = 0.006). Aortic root diameter 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). Pulse-wave velocity and flow-mediated dilatation were similar among the three groups.


CONCLUSIONS
Women with TS have greater IMT and conduit artery diameters than normal controls. Similarly, increased IMT in TS and 46,XX PA 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.
Ostberg
Julia E
JE
Department of Endocrinology, The Middlesex Hospital, Mortimer Street, London W1T 3AA, United Kingdom.
Donald
Ann E
AE
Halcox
Julian P J
JP
Storry
Clare
C
McCarthy
Carolyn
C
Conway
Gerard S
GS
D000328:Adult; D000568:Amenorrhea / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D001158:Arteries / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001783:Blood Flow Velocity; D001916:Brachial Artery / Q000000981:diagnostic imaging / Q000503:physiopathology; D002339:Carotid Arteries / Q000000981:diagnostic imaging / Q000503:physiopathology; D016022:Case-Control Studies; D004452:Echocardiography; D004548:Elasticity; D004730:Endothelium, Vascular / Q000503:physiopathology*; D005260:Female; D023961:Gonadal Dysgenesis, 46,XX / Q000150:complications; D006801:Humans; D010641:Phenotype; D011673:Pulsatile Flow; D017539:Tunica Intima / Q000000981:diagnostic imaging*; D014424:Turner Syndrome / Q000150:complications*; D014652:Vascular Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology*; D014664:Vasodilation