Impaired aortic distensibility and elevated central blood pressure in Turner Syndrome: a cardiovascular magnetic resonance study

Abstract

BackgroundWomen with Turner Syndrome have an increased risk for aortic dissection. Arterial stiffening is a risk factor for aortic dilatation and dissection. Here we investigate if arterial stiffening can be observed in Turner Syndrome patients and is an initial step in the development of aortic dilatation and subsequent dissection.MethodsFifty-seven women with Turner Syndrome (48 years [29–66]) and thirty-six age- and sex-matched controls (49 years [26–68]) were included. Distensibility, blood pressure, carotid-femoral pulse wave velocity (PWV), the augmentation index (Aix) and central blood pressure were determined using cardiovascular magnetic resonance, a 24-h blood pressure measurement and applanation tonometry. Aortic distensibility was determined at three locations: ascending aorta, transverse aortic arch, and descending aorta.ResultsMean aortic distensibility in the descending aorta was significantly lower in Turner Syndrome compared to healthy controls (P = 0.02), however, this was due to a much lower distensibility among Turner Syndrome with coarctation, while Turner Syndrome without coarctation had similar distensibility as controls. Both the mean heart rate adjusted Aix (31.4% vs. 24.4%; P = 0.02) and central diastolic blood pressure (78.8 mmHg vs. 73.7 mmHg; P = 0.02) were higher in Turner Syndrome compared to controls, and these indices correlated significantly with ambulatory night-time diastolic blood pressure. The presence of aortic coarctation (r = − 0.44, P = 0.005) and a higher central systolic blood pressure (r = − 0.34, P = 0.03), age and presence of diabetes were inversely correlated with aortic distensibility in TS.ConclusionAortic wall function in the descending aorta is impaired in Turner Syndrome with lower distensibility among those with coarctation of the aorta, and among all Turner Syndrome higher Aix, and elevated central diastolic blood pressure when compared to sex- and age-matched controls.Trial registrationThe study was registered at ClinicalTrials.gov (#NCT01678274) on September 3, 2012.Electronic supplementary materialThe online version of this article (10.1186/s12968-018-0497-0) contains supplementary material, which is available to authorized users.

Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years

“…Coarctation is associated with an increased likelihood of aortic dilation and dissection (Bambul Heck, Pabst von Ohain, Kaemmerer, Ewert, & Hager, ; Cools, Brown, & Gewillig, ; Eckhauser et al, ). Hypertension, a common consequence of aortic coarctation that may manifest in childhood, and altered compliance or distensibility of the aorta, which is prevalent in patients with coarctation, may both contribute to aortic disease (Hjerrild et al, ; Pees et al, ; Schoepp et al, ; Wen et al, ). Re‐interventions due to re‐coarctation are often necessary in adulthood.…”

Relationship between systolic hypertension assessed by 24‐hour ambulatory blood pressure monitoring and aortic diameters in young women with Turner syndrome

“…Although one study reported no association between BP and aortic growth during 2.4 years of follow‐up, several studies have reported hypertension to be an important and modifiable risk factor for aortic dilation . In particular, systolic BP was associated with aortic distensibility in patients with TS . According to the recent guideline for patients with TS, systolic hypertension is recommended as a target of treatment.…”

Journal of Cardiovascular Magnetic Resonance: 2017/2018 in review

“…Women with Turner Syndrome are at increased risk for aortic dissection. Wen et al [84] explored arterial stiffness in 57 women with Turner Syndrome. They found Turner Syndrome was associated with descending aorta distensibility, primarily among those with aortic coarctation, with similar distensibility among Turner Syndrome patients without coarctation.…”