Recommendations for Diagnostic Testing, Surveillance, and Surgical Intervention for Aortic Dilation in Turner Syndrome

Referenced studies that support the recommendations are summarized in the Online Data Supplement.

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.1–9
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 [m2]) is recommended to define the degree of aortic dilation and assess the risk of aortic dissection.9,10,11
1	C-LD	3. In patients with Turner syndrome without risk factors for aortic dissection (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.9,10,11
1	C-EO	4. In patients with Turner syndrome and an ASI >2.3 cm/m2, surveillance imaging of the aorta is recommended at least annually.9
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 (Figure 18).9
2a	C-LD	6. In patients with Turnery syndrome who are ≥15 years old and have an ASI of ≥2.5 cm/m2 plus risk factors for aortic dissection (Table 12), surgical intervention to replace the aortic root, ascending aorta, or both is reasonable.9,10
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.