Increased aortic stiffness in prepubertal girls with Turner syndrome

Groote, Katya De; Devos, Daniël; Herck, Koen Van; Wolf, Daniël De; Straaten, Saskia van der; Rietzschel, Ernst; Raes, Ann; Vandekerckhove, Kristof; Panzer, Joseph; Wilde, Hans De; Schepper, Jean De

doi:10.1016/j.jjcc.2016.03.006

Cited by 34 publications

(37 citation statements)

References 40 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Carotid-femoral PWV is an integrative measure that reflects the average arterial wall stiffness in the thoracic and abdominal aorta excluding the ascending aorta, the aortic arch and the adjacent descending aorta. Our data are in line with previous studies [45, 46] in the observation that carotid-femoral PWV is comparable between Turner Syndrome and matched controls, suggesting that this part of the aorta is not affected in Turner Syndrome. However, CMR studies evaluating this are lacking.…”

Section: Discussionsupporting

confidence: 93%

See 1 more Smart Citation

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 93%

“…Smaller studies in children and young adults with Turner Syndrome previously showed increased PWV and a greater Aix compared to controls [45, 46]. The present study finds that adults with Turner Syndrome have a raised Aix but comparable PWV.…”

Section: Discussionsupporting

confidence: 69%

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

View full text Add to dashboard Cite

show abstract

“…Old age and the presence of BAV are also associated with aortic diameters . 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 .…”

Section: Discussionmentioning

confidence: 99%

“…For all patients with TS, cardiac evaluation should be performed at diagnosis and regularly followed up at 3‐ to 5‐year intervals in adulthood to facilitate early detection of aortic disease . Patients with TS have a higher risk for aortic dilation due to inherent vasculopathy and/or oestrogen deficiency . According to a recent guideline, the use of Turner‐specific z ‐scores is recommended, particularly for those under 16 years old.…”

Section: Discussionmentioning

confidence: 99%

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

Lee

Kim

Lee

et al. 2019

Clinical Endocrinology

View full text Add to dashboard Cite

Objective: Patients with Turner syndrome (TS) are at high risk for cardiovascular morbidity and mortality due to aortic dilation. We evaluated the prevalence of hypertension and its risk factors and investigated the relationship between systolic hypertension and aortic diameter in young patients with TS. Design:Observational, cross-sectional study. Patients and measurements:Forty-two patients with TS (15-35 years) who had achieved final adult heights underwent 24-h ambulatory blood pressure monitoring (ABPM). Fasting glucose, insulin and lipid profiles were measured. The homeostasis model assessment of insulin resistance (HOMA-IR) was calculated. Echocardiography was performed to evaluate aortic diameters (aortic annulus, aortic root at the sinuses of Valsalva, sinotubular junction and ascending aorta), which were converted into Turner-specific z-scores.Results: Systolic and/or diastolic hypertension was identified in 71.4% (n = 30) of patients, as assessed by 24-hour ABPM. Twenty-eight patients (66.7%) were nondippers. Patients with systolic hypertension (n = 8, 19.0%) had a higher weight, waist circumference and HOMA-IR levels than those without hypertension (P < 0.05 for all).After adjusting for covariates, HOMA-IR was independently associated with systolic hypertension (odds ratio 10.1, P = 0.043). After adjusting for age and bicuspid aortic valve, systolic hypertension was independently related to increased aortic diameter at the aortic annulus (β = 1.064, P = 0.009) and sinotubular junction (β = 1.124, P = 0.016). Conclusions:Hypertension is highly prevalent and independently associated with IR in young patients with TS. The significant relationship between systolic hypertension and aortic diameters underscores the importance of BP and IR control. K E Y W O R D S ambulatory blood pressure monitoring, aortic diseases, hypertension, insulin resistance, Turner syndrome | 157 LEE Et aL.

show abstract

“…To our knowledge, this is the first broadly based investigation showing a significant correlation of deteriorated aortic elasticity values to TS only in a wide age range spectrum of pediatric TS patients, especially after exclusion of patients with aortic dilatation, CoA and BAVs. 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 ].…”

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

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.

show abstract

Increased aortic stiffness in prepubertal girls with Turner syndrome

Cited by 34 publications

References 40 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

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

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

Contact Info

Product

Resources

About