Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases

Objective: To describe a clinicopathologic study of a large group of congen itally bicuspid aortic valves surgically excised at a single institution. Material and Methods: The medical charts and bicuspid valves from patients undergoing aortic valve replacement at Mayo Clinic Rochester between 1991 a nd 1996 were retrospectively reviewed, Results: The age of the 542 patients ranged from 1 to 86 years (mean, 61), and 372 (69%) were men. Among these, 409 (75%) had pure aortic stenosis (AS ), 73 (13%) had pure aortic insufficiency (regurgitation) (AI), 53 (10%) ha d combined AS and AI, and 7(1%)had normal function. The mean age was higher for those with AS than AI (65 versus 46 years; P < 0.001), whereas the mal e-to-female ratio was higher for Al than AS (17.3:1 versus 1.7:1; P < 0.001 ). The two cusps were not equal in size in 95%, and a raphe was present in 76% (67% typical, 9% atypical). Raphal position was described in 315 and wa s between the right and left cusps in 270 (86%). Raphal absence occurred mo re often in valves with equal-sized cusps than unequal(33% versus 14%; P = 0.005), Moderate to severe calcification affected valves with AS more frequ ently than Al (99% versus 41%; P < 0.001), In contrast, annular dilatation was associated with Al more than AS ( 48% versus 11%; P < 0.001). Acquired commissural fusion involved valves with combined AS and Al mure often than the other functional states (31% versus 14%; P = 0.002), Sixteen patients ( age range, 18 to 78 years; 13 men) had infective endocarditis (6 active, 10 healed), including 10 with AI (9 men), 3 with AS plus Al, 2 with AS, and 1 with normal function but embolization. Conclusion: Functionally, the most common fate of congenitally bicuspid aor tic valves was calcific stenosis with or without regurgitation (85%). Becau se approximately 4 million C'S citizens have bicuspid valves and because va lve replacement is currently the only treatment of symptomatic AS, this dis order will continue to affect health-care costs.