PT  - JOURNAL ARTICLE
AU  - E J Turtle
AU  - A A Sule
AU  - D J Webb
AU  - L E Bath
TI  - Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations
AID  - 10.1136/archdischild-2014-307080
DP  - 2015 Jul 01
TA  - Archives of Disease in Childhood
PG  - 662--666
VI  - 100
IP  - 7
4099  - http://adc.bmj.com/content/100/7/662.short
4100  - http://adc.bmj.com/content/100/7/662.full
SO  - Arch Dis Child2015 Jul 01; 100
AB  - 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 remains underutilised, especially in children and adolescents, and there is currently little guidance on blood pressure (BP) assessment or hypertension management. Children and adolescents with TS at risk of dissection could be easily identified by timely imaging and BP assessment. This would allow medical management or surgical intervention to be put in place to reduce the risk of this major, and often fatal, complication. Since guidance is lacking, we have reviewed the literature on the risk factors for dissection in TS during childhood and adolescence, and make recommendations on the assessment and management of these patients.