Anatomically isolated aortic valve disease. Morphologic study of 100 cases at autopsy.

Anatomically isolated aortic valve disease accounted for 1.1% of all and 5.2% of cardiac autopsies over a 20 year period. Among a total of 100 symptomatic cases, 52% had congenitally bicuspid, 43% had tricuspid and 5% had congenitally unicuspid valves. Nineteen percent had undergone fibrous sclerosis, including 1% unicuspid, 3% tricuspid and 15% bicuspid valves. Thirty nine percent had been affected by infective endocarditis, including 20 bicuspid and 19 tricuspid valves. The remaining 42 showed fibrocalcification, including 4 unicuspid, 17 bicuspid and 21 tricuspid valves. The nature of the valvular disease showed a correlation with the age of the patient. Infective endocarditis, fibrosis and calcific disease occurred in an ascending age pattern, at average ages of around 30 years, mid to late thirties and mid to late forties, respectively. The lesions occurred much earlier on the bicuspid than on the tricuspid valves, except for infective endocarditis. The bicuspid deformity was not found to make the aortic valve more prone to infection, nor did infection occur earlier on it than on the tricuspid valve. Four of the 100 cases, all tricuspid, were considered to be of rheumatic origin, the reasons for which are discussed. Certain well established associated cardiac lesions were identified.