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The congenitally bicuspid aortic valve: A study of 85 autopsy cases

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Abstract

Clinical and necropsy observations are described in 85 autopsy cases in which the subjects had congenially bicuspid aortic valves. Sixty-one subjects had aortic stenosis with or without aortic regurgitation; 11, pure aortic regurgitation; and 13, unexpectedly, had a congenitally bicuspid aortic valve that apparently had functioned normally. The average age at death in patients with stenotic valves was 49 years, and in those with pure regurgitation, 31 years. Aortic valvular calcific deposits occurred in 59 of 61 patients with stenosis but was minimal or absent in the patients with pure regurgitation or normally functioning valves. Infective endocarditis appeared responsible for incompetency in 8 of 11 patients with pure regurgitation. Five of 61 patients with stenosis had had infective endocarditis, but infection occurred on a previously heavily calcified valve in only 1 patient.

Sixty-nine of the 72 patients with functionally abnormal valves were observed in a study of 400 autopsy cases; all subjects were over age 14 years, with severe valvular cardiac disease. The 13 functionally normal valves were found during study of 1,440 necropsy cases in which the subjects were free of other valvular or septal defects. The bicuspid aortic valve is the most frequent congenital malformation of the heart or great vessels. It occurs in over 50 percent of patients with anatomically isolated aortic stenosis and in about 25 percent of patients with clinically pure aortic regurgitation. It is not certain why 1 congenitally bicuspid valve becomes severely scarred, calcified and stenotic, another only mildly scarred and incompetent, another the site of infection, and why another remains free of these complications during life.

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