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6.1.2.5. Turner Syndrome: Diagnostic Testing, Surveillance,
and Surgical Intervention for Aortic Dilation in Turner
Syndrome
COR LOE
Recommendations
1 B-NR 1. In patients with Turner syndrome, TTE and cardiac MRI are
recommended at the time of diagnosis to evaluate for BAV,
aortic root and ascending aortic dilation, aortic coarctation,
and other congenital heart defects.
1 B-NR 2. In patients with Turner syndrome who are ≥15 years old, the
use of the ASI (ratio of aortic diameter [cm] to BSA [m
2
])
is recommended to define the degree of aortic dilation and
assess the risk of aortic dissection.
1 C-LD 3. In patients with Turner syndrome without risk factors for
aortic dissection (see Table 12), surveillance imaging with
TTE or MRI to evaluate the aorta is recommended every 5
years in children and every 10 years in adults, as well as before
planning a pregnancy.
1 C-EO 4. In patients with Turner syndrome and an ASI >2.3 cm/m
2
,
surveillance imaging of the aorta is recommended at least
annually.
1 C-EO 5. In patients with Turner syndrome and risk factors for aortic
dissection (Table 12), surveillance aortic imaging at an
interval depending on the aortic diameter, ASI, and aortic
growth rate is recommended (see Figure 18).
2a C-LD 6. In patients with Turner syndrome who are ≥15 years old
and have an ASI of ≥2.5 cm/m
2
plus risk factors for aortic
dissection (Table 12), surgical intervention to replace the
aortic root, ascending aorta, or both is reasonable.
2b C-EO In those without risk factors for aortic dissection, surgical
intervention to replace the aortic root, ascending aorta, or
both may be considered.
Table 12. Risk Factors for Aortic Dissection in Patients With
Turner Syndrome
Aortic coarctation
Aortic dilation
Bicuspid aortic valve
Hypertension
