COR LOE Recommendations Diagnostic I B-NR Adults with bicuspid aortic valve should be evaluated for coarctation of the aorta by clinical examination and imaging IIa B-NR It is reasonable to screen first-degree relatives of patients with bicuspid aortic valve or unicuspid aortic valve with echocardiography for valve disease and Therapeutic IIb B-NR In adults with bicuspid aortic valve stenosis and a noncalcified valve with no more than mild AR meeting indications for intervention it may be reasonable to treat with balloon valvuloplasty.S4.2.4-6 Synopsis Indications for aortic valve replacement according to generally apply. Recom- mendations above deal with issues specific to congen- ital aortic valve disease, which includes BAV, as well as unicuspid aortic valve and aortic stenosis caused by hy- poplastic aortic annulus. The underlying anatomy must be taken into account in patients with congenital aortic stenosis, as intervention may need to include annular enlarging procedures and other surgical techniques not commonly used in valvular aortic stenosis. These pa- tients are often young adults, for whom lifestyle consid- erations such as athletic endeavors, employment, and childbearing may influence the type of intervention. See Section 3.4 for recommendations on diagnos- tic evaluation; and Table 19 for routine testing and fol- low-up intervals. Recommendation-Specific Supportive Text 1. CoA has a male-to-female ratio of 1.5:1.S4.2.4-7 S4.2.4-13 A BAV is present in 50% to 70% of cases of CoA. Table 18. Subaortic Stenosis: Routine Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) *See Tables 3 and 4 for details on the ACHD AP classification system. 6-minute walk test or CPET, depending on the clinical indication. ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; SubAS, subaortic stenosis; and TTE, transthoracic echocardiogram. 