Background and aims of the study: This study reports the clinicopathol
ogic characteristics of congenital bicuspid aortic valves necessitatin
g surgical intervention. Methods: Among 374 aortic valve procedures du
ring the past 15 years, 63 cases of bicuspid aortic valve were encount
ered. Patients included 45 males and 18 females; mean age was 53 years
(range: 14 to 76 years). Results: Thirty-two patients demonstrated ao
rtic stenosis (AS), 27 aortic regurgitation (AR), and four had both co
nditions. Patients with AR were significantly younger than those with
AS (45 +/- 13 versus 60 +/- 11 years; p < 0.01). Histologic study of t
he excised valve disclosed severe myxoid degeneration in 16 of 27 pati
ents with AR. These patients underwent surgery at a younger age than t
he other 11 with calcification and endocarditis (40 +/- 13 versus 52 /- 10 years; p = 0.01). Based on the macroscopic appearance, the lesio
ns were divided into those with (n = 38) and without (n = 25) raphe. T
wenty-one patients (55%) in the former group demonstrated AR, whereas
19 (76%) in the latter group demonstrated AS. Patients with raphe were
significantly younger at the time of surgery than those without raphe
(50 +/- 15 versus 57 +/- 11 years; p = 0.02). As a complication, asce
nding aortic aneurysm was observed in six patients. Conclusions: In a
bicuspid aortic valve, calcification with stenosis commonly occurs in
most older patients. However, in some young adult patients with raphe,
AR may result front myxoid degeneration. The presence of raphe in the
bicuspid valve appears to have a significant influence on valve hemod
ynamics.