Critical aortic stenosis is associated with marked left ventricular myocardial hypertrophy. When the obstruction is hemodynamically significant, increased left ventricular systolic function occurs. Rarely. left ventricular systolic function can be decreased in childhood. In this paper, we described three children (aged 3 to 13) with severe aortic stenosis and heart failure. Each of the three patients had markedly reduced left ventricular systolic function as well as a dilated left ventricle with myocardial hypertrophy. The two cases who were operated showed clinical improvement.
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