Management of Cardiovascular Risks in Turner Syndrome

Cardiovascular Management

The American College of Cardiology recommends that all women with Turner syndrome undergo baseline cardiovascular imaging to evaluate for bicuspid aortic valve, coarctation of the aorta, and enlargement of the ascending aorta 1
The European Heart Journal suggests that the frequency of imaging should be determined by risk stratification, with low-risk women undergoing cardiology evaluation and TTE every 5-10 years, moderate-risk women every 2-5 years, and high-risk women annually 2, 3
CMR or CCT should be used if the ascending aorta is not adequately visualized by TTE, as recommended by the European Heart Journal 2
Prophylactic replacement of the aortic root or ascending aorta should be considered when the aortic size index (ASI) >23 mm/m² or the aortic height index (AHI) >23 mm/m, according to the European Heart Journal 2
Additional risk factors that warrant consideration for surgery include bicuspid aortic valve, coarctation of the aorta, uncontrolled hypertension, rapid aortic growth, and planned pregnancy, as noted in the European Heart Journal and Circulation 2, 4
Beta-blockers and/or ARBs may be considered to inhibit aortic growth, similar to management in Marfan syndrome, as suggested by the European Heart Journal 2, 3
Hypertension should be treated aggressively according to general guidelines, as recommended by the European Heart Journal 2, 3

A thorough cardiovascular evaluation is mandatory before attempting pregnancy, as noted in Circulation 6
Pregnancy is associated with a higher risk of aortic dissection, especially with pre-existing cardiovascular abnormalities, according to the Journal of the American College of Cardiology and Circulation 1, 6

The risk of aortic dissection is significantly higher than in the general population, as reported in Circulation 4, 5
Pregnancy in Turner syndrome carries substantial risks and requires extensive pre-conception counseling and monitoring, as recommended by the Journal of the American College of Cardiology 1
When assessing aortic dimensions, it's essential to use Turner-specific normative data or index measurements to body surface area or height, as noted in Circulation and the Journal of the American College of Cardiology 1, 6
Physical activity should be encouraged but modified based on cardiovascular status and aortic dimensions, as suggested by the European Heart Journal 2