Vascular dissection in women with Turner syndrome

Yetman, Angela T; Bisselou, Karl Stessy; Sanmann, Jennifer N.; Katz, Renee; Steingraeber, Catherine J.; Wilde, Megan; Murray, Mary; Starr, Lois J.

doi:10.1016/j.ijcard.2020.10.009

Cited by 7 publications

(6 citation statements)

References 14 publications

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“…The strict multidisciplinary monitoring of our population and the appropriate and timely treatment of hypertension and the other CV risk factors, such as diabetes, timely prophylactic surgical intervention for ascending aortic dilation (2.5% of cases in our population) or for coarctation of the aorta (3.7% of cases in our population), and, probably, the long-term and appropriate EPs therapy prevented the development of major CV events. In fact, despite the high incidence in our population of hypertension (23.8%) and of type 2 diabetes (16.2%), as well as the high prevalence of congenital heart defects predisposing patients to aortic dissection (bicuspid aortic valve 8.1%) [ 31 , 33 , 34 ], there was only one case of aortic dissection and no cases of myocardial infarction, stroke, or heart failure.…”

Section: Discussionmentioning

confidence: 96%

“…A possible explanation for this discrepancy is that our study population was on average younger at follow-up than the populations of the other two studies. However, the literature data show that lean and normotensive women with TS whose condition is also well controlled with hormone replacement therapy might never develop ischaemic heart disease, whereas hypertensive patients with TS and with insufficient hormone replacement therapy, obesity and type 2 diabetes are at very high risk of developing stroke or myocardial infarction [ 30 , 31 ].…”

Section: Discussionmentioning

confidence: 99%

“…As in the general population, hypertension is an important risk factor for the development of aortic dissection and myocardial infarction or stroke in TS [ 1 , 31 , 32 ]. The strict multidisciplinary monitoring of our population and the appropriate and timely treatment of hypertension and the other CV risk factors, such as diabetes, timely prophylactic surgical intervention for ascending aortic dilation (2.5% of cases in our population) or for coarctation of the aorta (3.7% of cases in our population), and, probably, the long-term and appropriate EPs therapy prevented the development of major CV events.…”

Section: Discussionmentioning

confidence: 99%

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New insights into the comorbid conditions of Turner syndrome: results from a long-term monocentric cohort study

Gambineri

Scarano

Rucci

et al. 2022

J Endocrinol Invest

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Purpose Many questions concerning Turner syndrome (TS) remain unresolved, such as the long-term complications and, therefore, the optimal care setting for adults. The primary aim of this long-term cohort study was to estimate the incidence of comorbid conditions along the life course. Methods A total of 160 Italian patients with TS diagnosed from 1967 to 2010 were regularly and structurally monitored from the diagnosis to December 2019 at the University Hospital of Bologna using a structured multidisciplinary monitoring protocol. Results The study cohort was followed up for a median of 27 years (IQR 12–42). Autoimmune diseases were the comorbid condition with the highest incidence (61.2%), followed by osteoporosis and hypertension (23.8%), type 2 diabetes (16.2%) and tumours (15.1%). Median age of onset ranged from 22 years for autoimmune diseases to 39 years for type 2 diabetes. Malignant tumours were the most prominent type of neoplasm, with a cumulative incidence of 11.9%. Papillary thyroid carcinoma was the most common form of cancer, followed by skin cancer and cancer of the central nervous system. Only one major cardiovascular event (acute aortic dissection) was observed during follow-up. No cases of ischaemic heart disease, heart failure, stroke or death were recorded. Conclusions This cohort study confirms the need for continuous, structured and multidisciplinary lifelong monitoring of TS, thus ensuring the early diagnosis of important comorbid conditions, including cancer, and their appropriate and timely treatment. In addition, these data highlight the need for the increased surveillance of specific types of cancer in TS, including thyroid carcinoma.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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New insights into the comorbid conditions of Turner syndrome: results from a long-term monocentric cohort study

Gambineri

Scarano

Rucci

et al. 2022

J Endocrinol Invest

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“…Affecting up to 1:2000 live-born females, Turner syndrome (TS) is a relatively common genetic disorder [ 1 ]. In addition to severe derangements of endocrine functions, congenital heart defects (CHD) occur in about 30% of patients [ 2 ] and half of the significantly increased risk of premature death is due to cardiovascular events [ 3 , 4 ]. Concerning CHD, bicuspid aortic valves (BAV; 30% [ 5 – 7 ]) and aortic coarctation (CoA; 15% [ 5 , 8 ]) — mostly co-occurring with BAV — are the most frequent forms, with higher risk of ascending aortic dilatation (AoD; 15–45%; [ 9 – 13 ]), starting at a relatively young age [ 14 ].…”

Section: Introductionmentioning

confidence: 99%

“…Concerning CHD, bicuspid aortic valves (BAV; 30% [ 5 – 7 ]) and aortic coarctation (CoA; 15% [ 5 , 8 ]) — mostly co-occurring with BAV — are the most frequent forms, with higher risk of ascending aortic dilatation (AoD; 15–45%; [ 9 – 13 ]), starting at a relatively young age [ 14 ]. The incidence of aortic dissection (AD) is increased 20- to 100-fold, taking place at a mean age of 35 years and not limited to individuals with previous aortic dilatation [ 2 , 15 – 17 ]. The underlying aortopathy in TS seems not to be restricted to dilated aortic segments, as implied by pathological elasticity changes of the ascending aorta, which are present as early as in childhood and adolescence [ 14 ].…”

Section: Introductionmentioning

confidence: 99%

Working towards risk stratification for ascending aortic dilatation in pediatric Turner syndrome patients: results of a longitudinal echocardiographical observation

Heno,

Michel-Behnke,

Pees

2023

Eur J Pediatr

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This study aimed to longitudinally evaluate aortic root dimensions and elasticity in pediatric Turner syndrome (TS) in relation to known cardiac implications such as coarctation of the aorta (CoA) and bicuspid aortic valves (BAV) in order to create an improved risk profile for the presumed underlying vessel pathology in childhood. We report on the longitudinal findings of our pediatric TS outpatient clinic over a period of up to 7.6 years. Forty-nine TS patients (median age at baseline 9.7 ± 5.9 years, range 0–19.8) were followed-up for on average 2.9 ± 1.1 examinations and a median time of 3.4 ± 1.6 years. Aortic root (AoR) diameters and corresponding Z-scores were determined echocardiographically, and elasticity parameters as well as annual progression rates were calculated. At baseline, 16.3% of patients showed Z-scores > 2 at one or more levels of the AoR (35.7% of patients with BAV, odds ratio of 4.2). There was net progression to be noted at all measuring levels, leading to 28.6% of patients (50% of patients with BAV) exhibiting aortic dilatation at the end of follow-up. Progression correlated with the presence of BAV, non-mosaic monosomy, and age. A levelling-off of progression was seen with the onset of adolescence.Conclusions: Marked progression of aortic diameters leading to the development of dilatation can be observed in TS patients during childhood and stresses the importance of close surveillance during childhood. Main risk factors are BAV and complete monosomy 45X0. A beneficial influence of estrogen substitution can be suspected but needs further investigation. What is known:• Patients with Turner syndrome are at an increased risk for aortic dilatation and dissection.• The presence of BAV and complete monosomy 45X are additional risk factors. What is new:• Aortic dilatation can be detected in pediatric patients with Turner syndrome.• Relevant progression in childhood is possible in at-risk individuals and warrants close surveillance.

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Aortic size predicts aortic dissection in Turner syndrome - A 25-year prospective cohort study

Thunström¹,

Thunström²,

Naessén³

et al. 2023

International Journal of Cardiology

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Vascular dissection in women with Turner syndrome

Cited by 7 publications

References 14 publications

New insights into the comorbid conditions of Turner syndrome: results from a long-term monocentric cohort study

New insights into the comorbid conditions of Turner syndrome: results from a long-term monocentric cohort study

Working towards risk stratification for ascending aortic dilatation in pediatric Turner syndrome patients: results of a longitudinal echocardiographical observation

Aortic size predicts aortic dissection in Turner syndrome - A 25-year prospective cohort study

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