Treatment of Turner Syndrome with Confirmed Hypertension and No Aortic Dilation
Clinical Scenario
This protocol applies to individuals with Turner syndrome who have confirmed hypertension without aortic dilation. The absence of aortic dilation is defined as: Z < 2.5 for those under 15 years; and aortic height index < 20 mm/m, aortic size index < 2.0 cm/m², or Z < 2.5 for those aged 15 years and older.
Key Comorbidity
Hypertension is the central focus of management in this subgroup. Current evidence supports treating hypertension according to standard paediatric or adult guidelines, adapted to the individual's age and any relevant co-existing conditions.
Confirmed hypertension
No aortic dilation
Treatment Approach (partial)
First-line antihypertensive therapy involves a specific class of agents that act on the renin–angiotensin system — the full selection and sequencing depend on age and any co-existing conditions.
Full regimen, dosing, and decision algorithm available in the complete protocol →
References
- DOI: 10.1093/ejendo/lvae050
- We recommend that medical treatment of hypertension for all individuals with TS who do not 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) should be based on the appropriate pediatric or adult guidelines for medical management of hypertension.
- If aortic disease is absent (TAV and Z < 2.5, AHI < 20 mm m⁻¹, or ASI < 2.0 cm m⁻², no dissection), initial treatment targets and antihypertensive medications should be based on current guidelines for hypertension, which recommend an angiotensin converting enzyme inhibitor or ARB as first-line therapy for adults and children, depending on co-existing conditions such as diabetes.
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