We report herein the results of a retrospective study conducted on ten cons
ecutive Japanese patients who underwent successful surgical relief of fixed
subaortic stenosis between 1972 and 1994 at ages ranging from 8 months to
21 Sears, and followed for 3.6 gears and 26 years, Associated cardiovascula
r defects were present in six patients, two had a history of infective endo
carditis, a discrete fibrous ring was found in nine patients, and a redunda
nt abnormal sheet was found in one. A stenotic structure was removed in nin
e patients and incised in one, while myotomy was additionally performed in
one, There were no early complications or deaths. Cardiac catheterization r
evealed a significant decrease in the peak systolic pressure gradient from
84 +/- 22 mmHg preoperatively to 32 +/- 22 mmHg postoperatively (P = 0,0017
), Reoperation of an aortic valve replacement with or without valvular annu
lus enlargement was required in four patients because of a small annulus wi
th aortic insufficiency or infective endocarditis. Infective endocarditis w
as a major cause of late mortality (n = 1) and morbidity (n = 1), but the r
emaining eight patients have been asymptomatic, Thus, although this lesion
is relatively rare in Japan, the typical discrete type may be more common t
han previously believed. While a relief operation is associated with low ea
rly mortality, the palliative aspect regarding pathology of the aortic valv
e should not be underestimated, including poor growth of the valve annulus,
deterioration of aortic insufficiency, and infective endocarditis, The mos
t appropriate operative procedure for reoperation remains to be evolved.