Recommendations for Replacement of the Aorta in Patients With Loeys-Dietz Syndrome

COR	LOE	Recommendations
1	C-LD	1. In patients with Loeys-Dietz syndrome and aortic dilation, the surgical threshold for prophylactic aortic root and ascending aortic replacement should be informed by the specific genetic variant, aortic diameter, aortic growth rate, extra-aortic features, family history, patient age and sex, and physician and patient preferences (Table 11).1–9
2b	C-EO	2. In patients with Loeys-Dietz syndrome attributable to a pathogenic variant in TGFBR1, TGFBR2, or SMAD3, surgery to replace the intact aortic arch, descending aorta, or abdominal aorta at a diameter of ≥4.5 cm may be considered, with the specific genetic variant, patient age, aortic growth rate, family history, presence of high-risk features (Table 11), and surgical risk informing the decision.