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

@article{Turtle2015AorticDI,
  title={Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations},
  author={Emma J. Turtle and Ashish Anil Sule and David John Webb and Louise E. Bath},
  journal={Archives of Disease in Childhood},
  year={2015},
  volume={100},
  pages={662 - 666}
}
There is a general lack of awareness of the risk of aortic dissection in Turner syndrome (TS) from both patients with TS and their physicians. Patients often ignore symptoms for up to 24 h before seeking medical advice, significantly increasing their risk of death. A clinical profile of those at risk of dissection is emerging and includes the presence of congenital heart defects, aortic dilatation and hypertension. MRI has revolutionised the visualisation of cardiovascular anatomy in TS but… 
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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 and at risk family members should be offered predictive testing if a mutation is identified.
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TLDR
A multidisciplinary approach addressing all aspects of their care including expert cardiological monitoring and intervention when required, access to fertility and obstetric expertise when appropriate, expert genetic counselling if indicated, and discussion of psychosocial, education, employment issues is key to the successful outcome for all women with Turner syndrome.
Cardiovascular risk in Turner syndrome.
  • B. Donato, M. J. Ferreira
  • Medicine
    Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
  • 2018
TLDR
The most common congenital cardiovascular defects are presented and illustrated with appropriate iconography and the current recommendations regarding the screening and monitoring of cardiovascular disease in patients with Turner syndrome are discussed.
Managing aortic aneurysms and dissections during pregnancy
TLDR
An overview of the underlying causes and risk factors for aortic aneurysms and dissections during pregnancy are provided, while presenting the ways of preventing and treating these conditions.
Managing aortic aneurysms and dissections during pregnancy
TLDR
An overview of the underlying causes and risk factors for aortic aneurysms and dissections during pregnancy are provided, while presenting the ways of preventing and treating these conditions.
Managing aortic aneurysms and dissections during
TLDR
An overview of the underlying causes and risk factors for aortic aneurysms and dissections during pregnancy are provided, while presenting the ways of preventing and treating these conditions.
Recognition and management of adults with Turner syndrome: From the transition of adolescence through the senior years
TLDR
This article focuses on the recognition and management of Turner syndrome from adolescents in transition, through adulthood, and into another transition as older women.
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References

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TLDR
In 11% of the cases, neither hypertension nor congenital heart disease were identified, suggesting that TS alone is an independent risk factor for aortic dissection; however, the cases where no risk factors were identified were very poorly documented.
Aortic dilatation in Turner syndrome: the role of MRI in early recognition
TLDR
MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals.
Clinical and epidemiological description of aortic dissection in Turner's syndrome
TLDR
Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life, and patients with Turner's Syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome.
Cardiovascular anomalies in Turner syndrome: spectrum, prevalence, and cardiac MRI findings in a pediatric and young adult population.
TLDR
The findings indicate that periodic cardiac MRI screening of persons with Turner syndrome is beneficial and the significant association between elongation of the transverse aortic arch and CoA, BAV, and aorti sinus dilatation may contribute to increased risk of aortIC dissection.
Cardiovascular Malformations and Complications in Turner Syndrome
TLDR
In the absence of structural cardiac malformations or HBP, the risk for aortic dissection appears small, and repeated echocardiography or magnetic resonance imaging to follow aorta root diameters does not appear to be warranted based on data currently available.
Turner Syndrome Is an Independent Risk Factor for Aortic Dilation in the Young
TLDR
Among young individuals with Turner syndrome, a bicuspid aortic valve predicts a larger proximal aorta, and growth-hormone use may predict a larger aorti annulus.
Aortic Dilatation and Dissection in Turner Syndrome
TLDR
The risk for aortic dissection is greatly increased in young women with Turner syndrome, and individuals with a dilated ascending aorta defined as aortsic size index >2.0 cm/m2 require close cardiovascular surveillance.
A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome.
TLDR
Reference ranges for absolute aortic dimensions in a TS population are presented to aid future interpretation of these measurements and two techniques are complementary and identify different aspects of cardiovascular pathology.
Moderate Aortic Enlargement and Bicuspid Aortic Valve Are Associated With Aortic Dissection in Turner Syndrome: Report of the International Turner Syndrome Aortic Dissection Registry
TLDR
Aortic dissection in Turner syndrome occurs in young individuals at smaller aortic diameters than in the general population or other forms of genetically triggered aortopathy.
Aortic dissection and rupture in a 16-year-old girl with Turner syndrome following previous progression of aortic dilation
TLDR
It is learned that rapid progression of aortic dilation should lead to immediate surgery to prevent more risky urgent intervention following the dissection of a 16-year-old girl with Turner syndrome.
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