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Idiopathic hypertrophic subaortic stenosis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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This is subvalvular aortic stenosis due to hypertrophic obstructive cardiomyopathy.

Left ventricular outflow obstruction occurs in some cases of hypertrophic cardiomyopathy with obstruction occurring only in mid-systole when the mitral valve is pulled towards the hypertrophied septum.

There is severe hypertrophy of the left ventricle (loud atrial (fourth) sound and abnormal ECG) and the pulse is sharp.

There is an ejection murmur later in systole than in valve stenosis but finishing before A2. There is no ejection sound and no diastolic murmur. The diagnosis is established by echocardiography.

Surgical relief of the obstruction is seldom indicated as the obstruction is part of a generalized disease of the heart muscle. However, if the symptoms are severe, a gutter can be cut through the septum on cardiopulmonary bypass or, rarely, the mitral valve may be replaced.


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