Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations

Turtle, Emma; Sule, Ashish Anil; Webb, David J.; Bath, Louise

doi:10.1136/archdischild-2014-307080

Cited by 26 publications

(14 citation statements)

References 34 publications

(52 reference statements)

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“…However, these methods may be less accurate when body surface area is less than 1.0 or greater than 2.5. Most individuals who develop aortic dissections have more than one of the following cardiovascular abnormalities: BAV, elongation of the transverse aorta, coarctation, hypertension, and/or an aortic aneurysm (Z > 3) (Carlson, Airhart, Lopez, & Silberbach, ; De Groote et al, ; Sybert, ; Turtle, Sule, Webb, & Bath, ).…”

Section: Cardiovascular Health Issuesmentioning

confidence: 99%

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

Lin

Prakash

Andersen

et al. 2019

American J of Med Genetics Pt A

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Turner syndrome is recognized now as a syndrome familiar not only to pediatricians and pediatric specialists, medical geneticists, adult endocrinologists, and cardiologists, but also increasingly to primary care providers, internal medicine specialists, obstetricians, and reproductive medicine specialists. In addition, the care of women with Turner syndrome may involve social services, and various educational and neuropsychologic therapies. This article focuses on the recognition and management of Turner syndrome

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Section: Cardiovascular Health Issuesmentioning

confidence: 99%

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

Lin

Prakash

Andersen

et al. 2019

American J of Med Genetics Pt A

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“…It was also notable that the number of cases of coarctation was statistically significantly higher than in non‐TS controls ( P = 0.03) (Mortensen et al, ). Furthermore, an association between coarctation and the dreaded aortic dissection has been mentioned by many (Roberts, ; Sybert, ; Turtle et al, ).…”

Section: Cardiovascularmentioning

confidence: 99%

Anatomy of turner syndrome

et al. 2016

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Turner syndrome (TS) is one of the most common sex chromosome abnormalities and results from total or partial monosomy of the X chromosome. It occurs in 1 in 2000 newborn girls and is also believed to be present in a larger proportion of conceptuses. There are various anatomic anomalies that have been associated with TS and the consequences of late recognition of these anomalies can be devastating. Aortic dilation and dissection occur at increased rates in TS patients and contribute to the decreased life expectancy of these patients. Such cases have prompted the need for early identification and continuous monitoring. Other anatomic variations increase morbidity in this population, and negatively impact the social and reproductive aspects of their lives. In this review, we summarize the cardiovascular, neurological, genitourinary, otolaryngolical, craniofacial, and skeletal defects associated with TS. To elucidate these morphological variations, novel illustrations have also been constructed. Clin. Anat. 29:638-642, 2016. © 2016 Wiley Periodicals, Inc.

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“…The incidence of bicuspid aortic valve (BAV) and coarctation of the aorta, as well as hypertension, are overrepresented in these women [11,12].…”

Section: Turner Syndrome (Ts)mentioning

confidence: 99%

Update on the Diagnosis and Management of Inherited Aortopathies, Including Marfan Syndrome

Zentner

West

Adès

2017

Heart, Lung and Circulation

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Key Points1. A number of inherited conditions can predispose the aorta, and less commonly other blood vessels, to dilatation and/ or rupture.2. Broadly speaking, these conditions are recognised as syndromic when accompanied by a number of systemic features or non-syndromic when the aortic dilatation appears to exist in isolation.3. The commonest syndromic aortopathy is Marfan syndrome and the commonest nonsyndromic aortopathy is that which accompanies congenital bicuspid aortic valve.4. Mutations in a number of genes have been identified, particularly in syndromic aortopathy.5. Although genotype-phenotype relationships exist, the phenotypes of the syndromic aortopathies may have significant overlap .6. When a syndromic aortopathy is suspected, review by a clinical geneticist is instrumental in characterising the clinical signs and the family history.7. Confirmation of a diagnosis (either clinically or by gene testing) allows identification of individuals at increased risk of aortic sequelae who will benefit from active medical management.8. Medical management is usually undertaken by a cardiologist with referral to other specialists (eg cardiothoracic surgeons) as appropriate.9. At risk family members should be offered predictive testing if a mutation is identified, and should otherwise be screened in keeping with the presumptive clinical diagnosis and assessment of risk.10. Pregnancy and the post-partum period confer a higher risk for aortic complications: a. Women with a personal or family history of aortopathy need appropriate preconception screening and counseling.b. Intervention may be required pre-conception and they should be managed closely throughout pregnancy, ideally in a high-risk obstetric clinic. A c c e p t e d M a n u s c r i p t 3 pregnancy, beta blockers can be used in pregnancy) and should include involvement of a cardiologist in the management and decision making for delivery.11. A clinical diagnosis of an inherited aortopathy can be made in the absence of a positive genetic test if the systemic features are consistent with a specific syndromic aortopathy. A familial history of aortic dissection in the absence of both a positive gene test and systemic examination findings may be more difficult to manage without a working clinical diagnosis. However, an inherited risk of dissection should nonetheless be considered in this setting, particularly if the process has affected young individuals and/or in the absence of traditional risk factors.

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Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations

Cited by 26 publications

References 34 publications

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

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

Anatomy of turner syndrome

Update on the Diagnosis and Management of Inherited Aortopathies, Including Marfan Syndrome

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