Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study

Devos, Daniël; Groote, Katya De; Babin, Danilo; Demulier, Laurent; Taeymans, Yves; Westenberg, Jos J.M.; Bortel, Luc Van; Segers, Patrick; Achten, Eric; Schepper, Jean De; Rietzschel, Ernst

doi:10.1186/s12968-017-0331-0

Cited by 26 publications

(22 citation statements)

References 51 publications

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“…Likewise, it is possible that the observed abnormalities of aortic distensibility are influenced by different blood flow profiles that has been demonstrated in Turner Syndrome, especially when BAV or CoA is present [43]. Contrary to the present study, a recent study of Turner Syndrome women found reduced aortic distensibility when BAV was present, which may possibly be due differences in the Turner Syndrome populations studied [44]. Here, we also show that the mean aortic area is similar or even slightly smaller (aortic arch) compared with controls, a difference that vanished after adjustment for BSA, but that the variability in aortic area is much larger, illustrated by the very wide range.…”

Section: Discussioncontrasting

confidence: 76%

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

Wen

Trolle

Viuff

et al. 2018

Journal of Cardiovascular Magnetic Resonance

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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.

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Section: Discussioncontrasting

confidence: 76%

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

Wen

Trolle

Viuff

et al. 2018

Journal of Cardiovascular Magnetic Resonance

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“…Another study showing pathological elasticity levels in TS children independent of their aortic valve morphology [ 24 ] excluded adolescents due to a suspected negative influence of HRT as well as children below 6 years of age because of lesser cooperation, presenting a study population of only 15 patients. The same group presented an MRI study verifying these findings in a bigger study cohort, but including adult patients in a great amount as well [ 25 ]. Therefore, our study represents a more complete and greater cohort of children and adolescents.…”

Section: Discussionmentioning

confidence: 83%

Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology

Pees¹,

Heno²,

Häusler

et al. 2018

Heart Vessels

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Turner syndrome (TS) is a common genetic disorder in females with high incidence of ascending aortic dilatation and even dissection occurring as early as in the second decade. Known risk factors (RF) are bicuspid aortic valves (BAV), coarctation of the aorta (CoA), and arterial hypertension. Since 10% of dissections occur in patients without RF, an intrinsic aortic wall abnormality has been postulated. This study aimed to investigate the elasticity of the ascending aorta as a surrogate marker of aortic wall texture. Forty-six pediatric patients with genetically proven TS were prospectively examined for the morphology of their aortic valve, and size and elasticity indices of the adjacent aorta. Cohorts of 46 female subjects with tricuspid aortic valves (TAV) and ten non-syndromic females with BAV were investigated as separate control groups. Comparison of healthy controls with TS patients revealed significantly deteriorated elasticity indices in those with TS. Furthermore, normalized aortic dimensions were greater in TS patients, but dilatations of the ascending aorta with z-score levels above two were restricted to those with BAV (14/46). Deteriorated elasticity indices were measured in TS patients, independent of aortic dilatation, BAV, and CoA, and were comparable to those of patients with isolated, non-syndromic BAVs. By measuring elasticity levels as a surrogate for aortic wall texture, we were able to gather evidence that TS presents with an intrinsic abnormality of the ascending aorta even in patients without concomitant BAV, CoA or dilatations as early as in childhood.

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“…Others have also investigated whether the presence of BAV can explain the increased aortic stiffness in patients with TS but the results are contradictory. Two studies 8,23 showed reduced ascending aortic distensibility and increased AA-PWV independently of the presence of BAV, while Devos et al 9 showed that only patients with BAV have a reduced ascending aortic distensibility. Our results are more in line with the first study, since we found no effect of BAV on stiffness.…”

Section: Discussionmentioning

confidence: 99%

Abnormal Aortic Wall Properties in Women with Turner Syndrome

et al. 2020

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Background Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS. Methods In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI). As control group, 13 women with BAV without TS and 48 healthy patients were included. Results Women with TS showed a higher AA-PWV (β = 1.08, confidence interval [CI]: 0.54–1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of elastin and diminished or absent expression of contractile proteins desmin, actin, and caldesmon, as well as the progesterone receptor. Conclusion Both aortic arch stiffness measurements on MRI and histomorphological changes point toward an inherent abnormal thoracic aortic wall in women with TS.

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Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study

Cited by 26 publications

References 51 publications

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

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

Aortic elasticity deterioration proves intrinsic abnormality of the ascending aorta in pediatric Turner syndrome unrelated to the aortic valve morphology

Abnormal Aortic Wall Properties in Women with Turner Syndrome

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