Aortic distensibility and dilation in Turner’s syndrome

Sharma, Jayendra; Friedman, Deborah; Dave-Sharma, Swati; Harbison, Madeleine D.

doi:10.1017/s1047951109990874

Cited by 31 publications

(20 citation statements)

References 17 publications

(41 reference statements)

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“…The natural history of aortic dilatation remains unknown in patients with TS. An intrinsic abnormality of elasticity in the ascending aorta has been suggested (23), involving disruption of the transforming growth factor b pathway (24,25).…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

Donadille

Rousseau

Zénaty

et al. 2012

European Journal of Endocrinology

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Objective: Congenital cardiovascular malformations and aortic dilatation are frequent in patients with Turner syndrome (TS). The objective of this study was to investigate the cardiovascular findings and management in a large cohort of patients, including children and adults. Design/methods: We recruited 336 patients with TS from a network of tertiary centers. We reviewed their files, checking for cardiovascular events, cardiac valve abnormalities, and aortic diameters indexed to body surface area (BSA) from magnetic resonance imaging (nZ110) or echocardiography (nZ300). Results: Informative cardiovascular data were available for only 233 patients. Vascular surgery was reported in 7.4% of the cohort. The first cause of surgery was aortic coarctation, detected in 6.9% at a median age of 9.5 (range: 0-60) years. Bicuspid aortic valve (BAV) was detected in 21% at a median age of 20 years (25th-75th percentiles: 15-30). At least one aortic diameter exceeded 32 mm in 12% of the cohort. This was detected at a median age of 19 (7-30) years. When indexed to BSA, at least one aortic diameter exceeded 20 mm/m 2 in 39% of the cohort. Conclusion: Our study shows that cardiovascular monitoring for TS patients is currently insufficient in France. BAV is present at birth, but often remains undiagnosed until later in life. Therefore, improved management in cardiovascular monitoring is required and a more systematic approach should be taken.

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Section: Discussionmentioning

confidence: 99%

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

Donadille

Rousseau

Zénaty

et al. 2012

European Journal of Endocrinology

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“…Aortic dissection can be a fatal complication during pregnancy in TS (20 -23, 25, 26). Recent studies have shown that aortic dissection in TS women is related not only to aortic valve disease and coarctation but also to aortopathy similar to that in Marfan syndrome with a collagen defect or cystic medial degeneration without macrovascular defects (35)(36)(37)(38). From a national survey and a literature review in the United States, Karnis and co-workers (25) estimated that the maternal risk of death from rupture or dissection of the aorta in pregnancy may be as high as 2% in TS.…”

Section: Discussionmentioning

confidence: 99%

Obstetric Outcomes in Women with Turner Karyotype

Hagman

Källén

Barrenäs

et al. 2011

The Journal of Clinical Endocrinology & Metabolism

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“…Aortic dilation in those without risk factors was accompanied by decreased aortic distensibility, suggesting an intrinsic abnormality in elastic property of the ascending aorta. Individuals with aortic dimensions in excess of the 95th percentile with decreased distensibility should be followed closely, both clinically and echocardiographically (84). One study demonstrated that CoA stenting is a safe and effective procedure at midterm follow-up in a small cohort of patients with TS.…”

Section: European Journal Of Endocrinologymentioning

confidence: 99%

“…The fact that death due to aortic dissection occurs in many younger TS women who have symptoms for O24 h before they seek medical attention emphasises the need to increase awareness of the critical significance of chest pain in young TS women (83). Aortic dissection can occur in TS without cardiac malformations or hypertension (83,84). Hypertension affects almost half of young adults with TS, and should therefore be closely monitored and treated vigorously (2).…”

Section: Cardiovascular Abnormalities In Transitionmentioning

confidence: 99%

TRANSITION IN ENDOCRINOLOGY: Treatment of Turner's syndrome during transition

Gawlik

Małecka-Tendera

2014

European Journal of Endocrinology

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Transition in health care for young patients with Turner's syndrome (TS) should be perceived as a staged but uninterrupted process starting in adolescence and moving into adulthood. As a condition associated with high risk of short stature, cardiovascular diseases, ovarian failure, hearing loss and hypothyroidism, TS requires the attention of a multidisciplinary team. In this review paper, we systematically searched the relevant literature from the last decade to discuss the array of problems faced by TS patients and to outline their optimal management during the time of transfer to adult service. The literature search identified 233 potentially relevant articles of which 114 were analysed. The analysis confirmed that all medical problems present during childhood should also be followed in adult life. Additionally, screening for hypertension, diabetes mellitus, dyslipidaemia, and osteoporosis is needed. After discharge from the paediatric clinic, there is still a long way to go.

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Aortic distensibility and dilation in Turner’s syndrome

Abstract: Individuals with Turner's syndrome, even without cardiovascular risk factors, do develop aortic dilation accompanied by decreased aortic distensibility, suggestive of an intrinsic abnormality in elastic property of the ascending aorta.

Cited by 31 publications

References 17 publications

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

Cardiovascular findings and management in Turner syndrome: insights from a French cohort

Obstetric Outcomes in Women with Turner Karyotype

TRANSITION IN ENDOCRINOLOGY: Treatment of Turner's syndrome during transition

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