Background: Morbidity and mortality from congenital and acquired cardiovascular (CV) disease is increased in Turner syndrome (TS), where traditional indices of CV risk are widely present but the single most common feature remains estrogen deficiency. Aim: To investigate CV risk in TS as expressed by the widely available ambulatory arterial stiffness index (AASI) and the impact of female sex hormone replacement therapy (HRT) hereon. Methods: TS women (n = 26) were examined following HRT washout and again during 6 months of HRT. Age-matched healthy female controls (n = 24) were examined once. 24-Hour ambulatory blood pressures, AASI in addition to metabolic and anthropometric indices of CV risk were measured. Results: The relatively tachycardic TS women had higher systolic and diastolic blood pressures. HRT reduced diastolic blood pressures with an increase in physical fitness, worsening of glucose tolerance, and a reduction in high-density lipoprotein. AASI was significantly elevated in TS when compared to controls (0.36 (0.02) vs. 0.26 (0.03), p = 0.01) but unaffected by HRT. Major explanatory variables to AASI were status (being TS or not), age, and diurnal pulse variability. Conclusion: AASI was elevated in TS, possibly indicating elevated CV risk with no impact of short-term HRT.

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