Turner syndrome
ICD-10 Q96.9 · ICD-11 LD50.0

Treatment of Turner Syndrome with Aortic Dilation

Aortic dilation in Turner syndrome (TS) is a cardiovascular finding that warrants prompt, structured pharmacological management. Age-stratified measurement thresholds determine when intervention is indicated, and treatment applies regardless of whether hypertension is also present.

Clinical scenario: Turner syndrome with aortic dilation — defined as a Z score ≥ 2.5 in individuals under 15 years of age; or, for those aged 15 years and older, an AHI ≥ 20 mm/m, ASI > 2.0 cm/m², or Z > 2.5.

Treatment approach: Management centres on pharmacological therapy from an established cardiovascular agent class — the full protocol specifies which agent or agents apply, the conditions under which each is selected, and how treatment is monitored over time.

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References

DOI: 10.1093/ejendo/lvae050

We recommend treatment with a beta-blocker, an angiotensin receptor blocker, or both for individuals with TS who have hypertension and have a dilated aorta (age < 15 years: Z ≥ 2.5; age ≥ 15 years: AHI ≥ 20 mm m−1, ASI > 2.0 cm m−2, or Z > 2.5).

We suggest that treatment with a beta-blocker, an angiotensin receptor blocker, or both should be considered for individuals with TS who have a dilated aorta (age < 15 years: Z ≥ 2.5; age ≥ 15 years: AHI ≥ 20 mm m−1, ASI > 2.0 cm m−2, or Z > 2.5), even if they are not hypertensive.

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