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10.1.1.2 Medical treatment In the absence of clinical trials, a pragmatic approach in a shared-decision model is adopted regarding TS medical treatment. Adoption of the strategy for inhibition of aortic growth with BBs and/or ARBs as in MFS may be considered. Hypertension should be treated according to general guidelines.300 Hormonal treatment with growth hormone (in childhood), sex (oestrogen and/or progesterone), and thyroid hormones needs to be discussed in a multidisciplinary team with the paediatrician and endocrinologist.1430–1434 10.1.1.3 Surgery of aortic aneurysms Aortic aneurysm surgery in TS should be informed, individualized, and consider factors beyond aortic diameter (indexed). These include BAV, coarctation, uncontrolled hypertension (despite more than three classes of antihypertensive drugs), rapid aortic growth (≥3 mm per year) and planned pregnancy. 10.1.1.4. Pregnancy and physical exercise Turner syndrome often involves fertility challenges, but assisted reproductive therapy has increased pregnancy rates. However, pregnancy in TS can elevate the risk of AD, particularly with additional risk factors (Figure 40). Recent studies suggest improved pregnancy outcomes due to better guideline adherence.1435,1436 Prophylactic aortic root surgery in women with TS contemplating pregnancy is recommended when the ASI reaches 25 mm/m2.1337 These decisions should be made by an expert team in a shared-decision process. Physical exercise has a beneficial impact on CVD risk and HRQoL in TS.1437 Structural congenital heart defects and aortic diameters (ASI, AHI and z-score) (Figure 40) need to be considered in the recommendations on the level of sports practice.1418 ASI AHI Z-score Risk group Low Moderate Moderate Moderate High High <20 mm/m2 ≥20–<23 mm/m2 ≥23–25 mm/m2 >25 mm/m2 >25 mm/m >4 ≥23–25 mm/m ≥20–<23 mm/m <20 mm/m 3.5–4 23 mm/m2 >23 mm/m2 >23 mm/m 23 mm/m - - >3.5 - Every 5–10 years: Cardiology evaluation and TTEb Every 3–5 years: Cardiology evaluation and TTEb Every 2–3 years: Cardiology evaluation and TTEb Every year: Cardiology evaluation and TTEb Consider surgery (Class IIb) Consider surgery (Class IIa) None of CoA, BAV, HTNa CoA, BAV, HTNa or aortic growth ≥3 mm/year Figure 40 Algorithm for surveillance in women (≥15 years) with Turner syndrome. AHI, aortic height index (ratio of aortic diameter [mm] to height [m]); ASI, aortic size index (ratio of aortic diameter [mm] to BSA [m2]); BAV, bicuspid aortic valve; BSA, body surface area; CCT, Cardiovascular Computed Tomography; CMR, cardiovascular magnetic resonance; CoA, coarctation of the aorta; HTN, arterial hypertension; TTE, transthoracic echocardiography. aHTN: arterial hypertension, not under control despite more than three classes of antihypertensive drugs. bCMR (preferably) or CCT if inadequate visualization of the ascending aorta. Recommendation Table 58 — Recommendations for aortic surgery in women with Turner syndrome Recommendations Classa Levelb Elective surgery for aneurysms of the aortic root and/ or ascending aorta should be considered in women with TS who are ≥15 years of age, have an ascending ASI >23 mm/m2, an AHI >23 mm/m, a z-score >3.5, and have associated risk factors for aortic dissectionc or are planning pregnancy.70,1417,1421 IIa C Continued Elective surgery for aneurysms of the aortic root and/ or ascending aorta may be considered for women with TS who are ≥15 years of age, have an ascending ASI >25 mm/m2, an AHI >25 mm/m, a z-score >4, and who do not have associated risk factors for aortic dissectionc.70,1417,1421 IIb C © ESC 2024 AHI, aortic height index (ratio of aortic diameter [mm] to height [m]); ASI, aortic size index (ratio of aortic diameter [mm] to BSA [m2]); TS, Turner syndrome. aClass of recommendation. bLevel of evidence. cBicuspid aortic valve, elongation of the transverse aorta, coarctation of the aorta, and/or uncontrolled hypertension (despite more than three classes of antihypertensive drugs). See Figure 40. Downloaded from academic.oup.com/eurheartj/article/45/36/3538/7738955 by guest on 07 October 2026