Differences in Distribution of Myocardial Abnormalities in Patients with Obstructive and Nonobstructive Asymmetric Septal Hypertrophy (ASH)

Abstract

Patients with typical idiopathic hypertrophic subaortic stenosis (IHSS) represent only one subgroup of a cardiac disease in which the characteristic anatomic abnormality is asymmetric septal hypertrophy (ASH). In most patients with ASH, left ventricular outflow obstruction is absent and cardiac dysfunction presumably is due to widespread involvement of the left ventricle by an underlying myocardial abnormality. In other patients with ASH, left ventricular outflow obstruction is present (typical IHSS) and constitutes a major feature of the hemodynamic and physical findings. To determine whether patients with outflow obstruction also have the underlying myocardial abnormality diffusely involving the left ventricle, the gross morphology of hearts from patients with and without outflow obstruction were studied both by necropsy and by echocardiography. Echocardiographic studies revealed that the ventricular septum was thicker in obstructive ASH, a finding confirmed by the postmortem studies. The necropsy studies also indicated that although the left ventricular free wall was thickened in both obstructive and nonobstructive ASH, the configuration of the left ventricular free wall was distinctly different in the two groups. In obstructive ASH, the free wall was hypertrophied and identical in appearance to that seen in valvular aortic stenosis. Moreover, echocardiographic studies indicated that the thickening of the free wall behind posterior mitral leaflet appeared to regress after operative relief of the outflow obstruction. In contrast, the left ventricular free wall of severely symptomatic patients without outflow obstruction had a markedly different and unique appearance; the free wall of left ventricle directly behind the posterior mitral leaflet was of normal or less than normal thickness, whereas the remaining free wall was nonuniformly thickened. On the basis of these findings and the microscopic data presented in the companion paper, we conclude that the myocardial abnormality in obstructive ASH (typical IHSS) is localized largely to the ventricular septum, with left ventricular free wall thickening occurring as a consequence of outflow obstruction. In symptomatic patients with nonobstructive ASH, however, the data suggest that the left ventricle, including free wall, is extensively involved with a primary myocardial abnormality.