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Hereditary Aortopathy

Turner Syndrome Cardiovascular Pathway

Turner-specific aortic measurement, AHI and ASI assessment, risk modifiers and guideline-linked cardiovascular surveillance.

Clinical pathway

Turner syndrome aortopathy surveillance

Web pathway

For age ≥15 years, AHI and ASI are calculated from the ascending aortic diameter. For age <15 years, the TS-specific Z-score remains clinician-entered because no exact validated model is verified in the current library.

A

Hereditary aortopathy

How to measure the aortic root

Standardised measurement technique is essential for serial assessment of hereditary aortopathy. Measurements should be performed consistently and according to the convention used by the selected reference dataset.

Three-dimensional drawing of the aortic root in the parasternal long-axis view. Four numbered diameters are drawn perpendicular to the long axis of the root: 1, the aortic annulus, hinge point to hinge point of the aortic leaflets; 2, the sinuses of Valsalva at their widest; 3, the sinotubular junction, where the sinuses meet the tubular aorta; 4, the proximal ascending aorta above the junction.
Where to measure the aortic root, parasternal long-axis view. Cardiac phase and edge convention follow the selected reference model.
  • Use the parasternal long-axis view for TTE assessment.
  • Measure perpendicular to the long axis of the vessel.
  • Use the phase and edge convention required by the selected reference dataset, and keep the technique consistent across serial studies.
  • If TTE is inadequate, cross-sectional imaging may be required.
01

Patient / assessment inputs

Body-size and aortic measurements

BSA is calculated with the Haycock method: 0.024265 × height(cm)^0.3964 × weight(kg)^0.5378. Current BSA: —. Z-score change is calculated only when both valid serial Turner-specific Z-scores and valid dates are supplied.

02

Reference model status

Turner-specific indexing

Automatic Turner-specific Z-score: not available

No exact, citation-backed Turner-specific model is verified in the current library. No substitute model is used.

03

Results

Aortic measurements and indexing

Aortic measurementsIncomplete
Body-size / indexing resultIncomplete
Degree of aortic dilationEnter age, height, weight and ascending aorta
Change from previous studyNo serial result
04

Clinical context

Risk modifiers

Turner guideline rapid-growth modifier: >3 mm/year. For pediatric serial Turner-specific Z-scores, >1 Z/year is also important. Exact equality is not flagged. The mm/year figure compares the previous measurement with the current measurement of the selected segment only.

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Guideline-linked context

Surveillance and specialist review

Imaging surveillance

Establish a baseline cardiovascular assessment and use serial echocardiography when appropriate. Consider more frequent imaging when dilation, rapid growth, or other high-risk features are present.

Use CMR or CT when TTE is inadequate or when assessment of the distal aorta is needed.

Clinical management

Blood-pressure surveillance and management, individualised activity advice, and review by an experienced aortopathy team are important. Medical therapy and exercise decisions require the treating team’s context.

Give particular attention to BAV, coarctation, hypertension, rapid growth, and adolescent/adult transition planning.

Interpretation boundary

These pathway outputs support structured review. They do not issue an automatic surgical or “operate” recommendation. Findings meeting a guideline threshold should prompt specialist aortic surgical assessment.

References and provenance

Gravholt CH et al. Clinical Practice Guidelines for the Care of Girls and Women With Turner Syndrome. Eur J Endocrinol. 2024;190(6):G53–G151. DOI: 10.1093/ejendo/lvae050

BSA: Haycock GB, Schwartz GJ, Wisotsky DH. J Pediatr. 1978;93(1):62–66. PubMed

Developed by Dr Michael Sammaan · Paediatric Cardiology · Advanced Echocardiography & Cardiac CT · Evidence reviewed: September 2026

For healthcare professional use. Guideline interpretation should be individualised and applied alongside local specialist practice. ZED Cardio does not replace specialist clinical judgement.