BAV and Associated Aortopathy 5.1.1. Diagnosis and Follow-Up of BAV 5.1.1.1. Diagnostic Testing: Initial Diagnosis Recommendations for Diagnostic Testing: Initial Diagnosis of BAV Referenced studies that support the recommendations are summarized in Online Data Supplement 18. COR LOE Recommendations In patients with a known BAV, TTE is indicated to evaluate valve morphology, measure severity of AS and AR, assess the shape and diameter of the aortic sinuses and ascending aorta, and evaluate for the presence of aortic coarctation for prediction of clinical outcome and to determine timing In patients with BAV, CMR angiography or CT angiography is indicated when morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography.4,5 In first-degree relatives of patients with a known BAV, a screening TTE might be considered to look for the presence of a BAV or asymptomatic dilation of the aortic sinuses and ascending aorta.6 Synopsis BAV is a common congenital anomaly that affects 0.5% to 2.0% of adults with a 3:1 male-to-female predomi- Patients with BAV may develop isolated aortic valve disease, including isolated AR, AS, or a combina- tion of the two. Aortic aneurysms have been reported in 20% to 40% of patients with BAV.1 This aortopathy can occur independent of valve function and consists of dilation of the aortic sinuses, the ascending aorta, or the arch. Therefore, patients with BAV require care- ful evaluation of both the aortic valve and the aorta throughout their lifetimes (Figure 5). Recommendation-Specific Supportive Text 1. Many patients with BAV will develop AS or AR over their lifetimes. 