Recommendations for BAV Aortopathy

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

COR	LOE	Recommendations
1	B-NR	1. In patients with a BAV, TTE is indicated to evaluate valve morphology and function, to evaluate the diameter of the aortic root and ascending aorta, and to evaluate for aortic coarctation and other associated cardiovascular defects.1–4
1	C-LD	2. In patients with a BAV, CT or MRI of the thoracic aorta is indicated when the diameter and morphology of the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE.1
1	C-LD	3. In patients with a BAV and either HTAD or phenotypic features concerning for Loeys-Dietz syndrome, a medical genetics evaluation is recommended.5,6
1	C-LD	4. In patients with a BAV and a dilated aortic root or ascending aorta, screening of all first-degree relatives by TTE is recommended to evaluate for the presence of a BAV, dilation of the aortic root and ascending aorta, or both; if the diameter and morphology of the aortic root, ascending aorta, or both cannot be assessed accurately or completely by TTE, a cardiac-gated CT or MRI of the thoracic aorta is indicated.7
2a	B-NR	5. In patients with a BAV, screening of all first-degree relatives by TTE is reasonable to evaluate for the presence of a BAV, dilation of the aortic root and ascending aorta, or both.7–10