Cardiovascular Health in Turner Syndrome: A Scientific Statement From the American Heart Association

Circ Genom Precis Med. 2018 Oct;11(10):e000048. doi: 10.1161/HCG.0000000000000048.


Girls and women with Turner syndrome face a lifelong struggle with both congenital heart disease and acquired cardiovascular conditions. Bicuspid aortic valve is common, and many have left-sided heart obstructive disease of varying severity, from hypoplastic left-sided heart syndrome to minimal aortic stenosis or coarctation of the aorta. Significant enlargement of the thoracic aorta may progress to catastrophic aortic dissection and rupture. It is becoming increasingly apparent that a variety of other cardiovascular conditions, including early-onset hypertension, ischemic heart disease, and stroke, are the major factors reducing the life span of those with Turner syndrome. The presentations and management of cardiovascular conditions in Turner syndrome differ significantly from the general population. Therefore, an international working group reviewed the available evidence regarding the diagnosis and treatment of cardiovascular diseases in Turner syndrome. It is recognized that the suggestions for clinical practice stated here are only the beginning of a process that must also involve the establishment of quality indicators, structures and processes for implementation, and outcome studies.

Keywords: AHA Scientific Statements; Turner syndrome; aortic aneurysm; genetics; heart diseases; pregnancy.

Publication types

  • Review

MeSH terms

  • American Heart Association
  • Aortic Coarctation* / diagnosis
  • Aortic Coarctation* / pathology
  • Aortic Coarctation* / physiopathology
  • Aortic Coarctation* / therapy
  • Aortic Dissection* / diagnosis
  • Aortic Dissection* / pathology
  • Aortic Dissection* / physiopathology
  • Aortic Dissection* / therapy
  • Aortic Valve / abnormalities
  • Aortic Valve / pathology
  • Aortic Valve / physiopathology
  • Bicuspid Aortic Valve Disease
  • Female
  • Heart Defects, Congenital* / diagnosis
  • Heart Defects, Congenital* / pathology
  • Heart Defects, Congenital* / physiopathology
  • Heart Defects, Congenital* / therapy
  • Heart Valve Diseases / pathology
  • Heart Valve Diseases / physiopathology
  • Heart Valve Diseases / therapy
  • Humans
  • Hypertension* / diagnosis
  • Hypertension* / pathology
  • Hypertension* / physiopathology
  • Hypertension* / therapy
  • Turner Syndrome* / diagnosis
  • Turner Syndrome* / pathology
  • Turner Syndrome* / physiopathology
  • Turner Syndrome* / therapy
  • United States