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Aortic dissection in children and adolescents with Turner syndrome: risk factors and management recommendations
  1. E J Turtle1,
  2. A A Sule2,
  3. D J Webb3,
  4. L E Bath4
  1. 1Edinburgh Centre for Endocrinology, Western General Hospital, Edinburgh, UK
  2. 2Tan Tock Seng Hospital, Singapore, Singapore
  3. 3Queen's Medical Research Institute, University of Edinburgh/BHF Centre for Cardiovascular Science, Edinburgh, UK
  4. 4Edinburgh Centre for Endocrinology, Royal Hospital for Sick Children, Edinburgh, UK
  1. Correspondence to Dr Emma Turtle, Edinburgh Centre for Endocrinology, Western General Hospital, Metabolic Unit, Crewe Road South, Edinburgh EH4 2XU, UK; emma.turtle{at}ed.ac.uk

Abstract

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.

  • Endocrinology
  • Adolescent Health
  • Congenital Abnorm
  • Cardiology

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