SPECIAL ARTICLES Asymmetric Septal Hypertrophy (ASH): The Unifying Link in the IHSS Disease Spectrum
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چکیده
IN THE EARLY 1960s, a distinctive cardiac disease was recognized and named obstructive cardiomyopathy in the United Kingdom,' idiopathic hypertrophic subaortic stenosis (IHSS) in the United States,2 and muscular subaortic stenosis in Canada.3 Each name emphasized left ventricular outflow obstruction, the feature thought to be characteristic of the disease. Subsequently, it was recognized that a large number of patients who had many of the symptoms, physical findings, and angiographic features of IHSS demonstrated little or no left ventricular outflow gradient.4-1" The concept thus evolved that patients with obstruction represent only one part of a spectrum of disease in which outflow obstruction may be severe, mild, or even absent. Moreover, since patients without obstruction may nonetheless have severe symptoms, some clinicians consider inflow obstruction, due to severely diminished left ventricular compliance, as the major determinant of cardiac dysfunction.7 10-12 The above considerations clearly indicate that present terminology, emphasizing left ventricular outflow obstruction, is imprecise.'2-16 The term hypertrophic cardiomyopathy (with or without obstruction) has been proposed as a more accurate name.'0 This terminology distinguishes the IHSS disease spectrum from dilated (congestive) cardiomyopathy, but not from left ventricular hypertrophy of other etiology (such as hypertensive heart disease, infiltrative cardiomyopathies, and amyloid heart disease).
منابع مشابه
Asymmetric septal hypertrophy (ASH): the unifying link in the IHSS disease spectrum. Observations regarding its pathogenesis, pathophysiology, and course.
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Systolic anterior motion of the mitral valve in the absence of asymmetric septal hypertrophy.
Asymmetric septal hypertrophy (ASH) is considered the unifying link in the spectrum of hypertrophic cardiomyopathies. ASH and mitral valve systolic anterior motion (SAM) are the two most important echocardiographic criteria for the diagnosis of idiopathic hypertrophic subaortic stenosis (IHSS). Ten patients found have SAM without ASH were studied. Septal thickness, thickening, and excursion wer...
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Idiopathic hypertrophic subaortic stenosis (IHSS) is characterized by subaortic obstruction to left ventricular (LV) outflow. However, the obstruction is variable and many patients have no resting gradient and no clinical evidence of IHSS. Although a characteristic systolic movement of the anterior mitral valve leaflet can be demonstrated echocardiographically in many patients with IHSS, this m...
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The specificity and significance of the asymmetric septal hypertrophy (ASH) and myocardial fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS) is uncertain. To examine this we studied 215 hearts, including normal embryos, fetuses, children, and adults; and hearts with congenital and acquired disease. Disproportionate septal thickening was present in all embryos and in some abnor...
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Previous studies have shown that asymmetric septal hypertrophy (ASH) may occur in embryonic and malformed hearts and that muscle cell disarray is found in myocardium that contracts isometrically. To account for the characteristic ASH and septal muscle fiber disarray of idiopathic hypertrophic subaortic stenosis (IHSS), we postulated that a catenoid shape of the septum, i.e., net zero curvature,...
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تاریخ انتشار 2005