Echocardiographic Pseudo Idiopathic Hypertrophic Subaortic Stenosis

Abstract

chocardiography has been demonstrated to be a E very sensitive and specific noninvasive diagnostic method for the evaluation of patients with idiopathic hypertrophic subaortic stenosis ( IHSS ) It has been suggested that asymmetric hypertrophy of the left ventricle, with the interventricular septum at least 1.3 times as thick as the left ventricular free wall as identified by echocardiography, is the pathognomonic anatomic abnormality of IHSS.3.7 This finding, in conjunction with the characteristic systolic anterior motion of the anterior leaflet of the mitral valve which can also be identified by echocardiography, has been thought to be specific for the diagnosis of IHSS. The purpose of this report is to present a case of primary pulmonary hypertension simulating IHSS by the echocardiographic criteria mentioned above. on chest x-ray film. There was evidence of mild obstructive lung disease by pulmonary function tests. Cardiac catheterization revealed pulmonary hypertension with a systolic pulmonary artery pressure of 100 mm Hg. There was no evidence for an intracardiac shunt. There was no pressure gradient within the left ventricle either at rest or during provocative maneuvers and no evidence of mitral stenosis. Large central pulmonary arteries with rapid tapering of their branches was demonstrated by angiography. No evidence for pulmonary emboli was seen. Forward angiogram showed no evidence for IHSS. An echocardiogram obtained during hospitalization (Fig 1 ) showed asymmetric septal hypertrophy with gross thickening of the interventricular septum. In systole a distinct anterior motion of the anterior mitral ledet began soon after the onset of systole and returned to meet the posterior ledet before the end of systole. The right ventricular dimension ( not shown well in Fig 1 ) was enlarged.