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J Heart Valve Dis. 1998 Sep;7(5):500-3.

Congenital bicuspid aortic valve: analysis of 63 surgical cases.

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  • 1Second Department of Surgery, Kagoshima University, Faculty of Medicine, Japan.



This study reports the clinicopathologic characteristics of congenital bicuspid aortic valves necessitating surgical intervention.


Among 374 aortic valve procedures during the past 15 years, 63 cases of bicuspid aortic valve were encountered. Patients included 45 males and 18 females; mean age was 53 years (range: 14 to 76 years).


Thirty-two patients demonstrated aortic stenosis (AS), 27 aortic regurgitation (AR), and four had both conditions. Patients with AR were significantly younger than those with AS (45 +/- 13 versus 60 +/- 11 years; p < 0.01). Histologic study of the excised valve disclosed severe myxoid degeneration in 16 of 27 patients with AR. These patients underwent surgery at a younger age than the other 11 with calcification and endocarditis (40 +/- 13 versus 52 +/- 10 years; p = 0.01). Based on the macroscopic appearance, the lesions were divided into those with (n = 38) and without (n = 25) raphe. Twenty-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, ascending aortic aneurysm was observed in six patients.


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 from myxoid degeneration. The presence of raphe in the bicuspid valve appears to have a significant influence on valve hemodynamics.

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