Evidence for muscle fiber hyperplasia in the septum of patients with hypertrophic obstructive cardiomyopathy (HOCM). Quantitative examination of endomyocardial biopsies (EMCB) and myectomy specimens
Abstract
In hypertrophic cardiomyopathy, muscle mass is increased for no apparent reason (6). A familial occurrence in 20% of the cases indicates a genetic disposition. The most important feature of HOCM is a thickening of the septum disproportionate to the hypertrophied free left ventricular wall, whereas the cavity remains normal or is narrowed (14, 18). The catenoid-shaped septum bulges into the outflow tract of the left ventricle and results in its systolic obstruction (8). This obstruction is mainly located in the subaortic region, rarely in the middle or apical parts of the left ventricle (9). The disorganization of muscle fibers is the second hallmark of HOCM. Fiber disarray can be observed in various heart diseases as well as in normal hearts (1, 2, 17, 20), but when its distribution is diffuse (4) and quantitatively substantial (11, 12) it is quite specific to hypertrophic cardiomyopathy.
Keywords
Hypertrophic Cardiomyopathy Volume Density Endomyocardial Biopsy Normal Shape Hypertrophic Obstructive CardiomyopathyPreview
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References
- 1.Becker AE, Caruso G (1982) Myocardial disarray: A critical review. Br Heart J 47: 527–538Google Scholar
- 2.Bulkley BH, Weisfeldt M, Hutchins GM (1977) Asymmetric septal hypertrophy and myocardial fiber disarray: features of normal developing and malformed hearts. Circulation 56: 296–298Google Scholar
- 3.Ferrans VJ, Rodriguez ER (1983) Specifity of light and electron microscopic feature of hypertrophia obstructive and nonobstructive cardiomyopathy. Qualitative, quantitative and etiologic aspects Eur Heart J 4, Suppl 7: 2–22Google Scholar
- 4.Fujiwara H, Hoshino T, Fujiwara T, Kawai C, Hamashima Y (1982) Classification and distribution of myocardial fascicle and fiber disarray in 14 hearts with hypertrophie cardiomyopathy in 25 µ thick sections. Jpn Circ J 46: 225–234PubMedCrossRefGoogle Scholar
- 5.Fujiwara H, Hoshino T, Yamana K, Fujiwara T, Furuta M, Hamashima Y, Kawai C (1983) Number and size of myocytes and amount of interstitial space in the ventricular septum and in the left ventricular free wall in hypertrophie cardiomyopathy. Am J Cardiol 52: 818–823PubMedCrossRefGoogle Scholar
- 6.Goodwin JF (1970) Congestive and hypertrophic cardiomyopathies. A decade of study. Lancet i: 731–739Google Scholar
- 7.Hoshino T, Fujiwara H, Kawai C, Hamashima Y (1983) Myocardial fiber diameter and regional distribution in the ventricular wall of normal adult hearts, hypertensive hearts and hearts with hypertrophie cardiomyopathy. Circulation 67: 1109–1116PubMedCrossRefGoogle Scholar
- 8.Hutchins GM, Bulkley BH (1978) Catenoid shape of the intervenricular septum: possible cause of idiopathic hypertrophie subaortic stenosis. Circulation 58: 392–397PubMedGoogle Scholar
- 9.Kuhn H, Mercier J. Köhler E, Frenzel H, Hort W, Loogen F (1983) Differential diagnosis of hypertrophic cardiomyopathies: typical (subaortic) hypertrophic obstructive cardiomyopathy, atypical (mid-ventricular) hypertrophic obstructive cardiomyopathy and hypertrophic non-obstructive cardiomyopathy. Eur Heart J 4, Suppl F: 93–104Google Scholar
- 10.Loud AV, Anversa P (1984) Biology of disease. Morphometric analysis of biologic process. Lab Invest 50: 250–261Google Scholar
- 11.Maron BJ, Roberts WC (1979) Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophie cardiomyopathy. Circulation 59: 689–706PubMedGoogle Scholar
- 12.Maron BJ, Epstein SE (1980) Hypertrophic cardiomyopathy: Recent observations regarding the specifity of three hallmarks of the disease: asymmetric septal hypertrophy, septal disorganization and systolic anterior motion of the anterior mitral leaflet. Am J Cardiol 45: 141–154PubMedCrossRefGoogle Scholar
- 13.Maron BJ, Spirito P, Wesley Y, Arce J (1986) Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. N Engl J Med 315: 610–614PubMedCrossRefGoogle Scholar
- 14.Maron BJ, Wolfson JK, Epstein SE, Roberts WC (1986) Intramural (“small vessel”) coronary artery disease in hypertrophie cardiomyopathy. J Am Coll Cardiol 8: 545–547PubMedCrossRefGoogle Scholar
- 15.Olsen EGJ (1980) The pathology of idiopathic hypertrophic subaortic stenosis (hypertrophie cardiomyopathy). A critical review. Am Heart J 100: 553–562PubMedCrossRefGoogle Scholar
- 16.Rakusan K (1984) Cardiac growth, maturation, and aging. In: Zak R (ed) Growth of the Heart in Health and Disease, Raven Press, New York, pp 131–164Google Scholar
- 17.Roberts WC, Ferrans VJ (1975) The pathologic anatomy of the cardiomyopathies (idiopathic dilated and hypertrophic types, infiltrative types and endomyocardial disease with and without eosinophilia). Human Pathol 6: 287–342CrossRefGoogle Scholar
- 18.Teare D (1958) Asymmetrical hypertrophy of the heart in young adults. Br Heart J 20: 1–8PubMedCrossRefGoogle Scholar
- 19.Unverferth DV, Baker PB, Swift SE, Chaffee R, Fetters IK, Uretsky BF, Thompson ME, Leier CV (1986) Extent of myocardial fibrosis and cellular hypertrophy in dilated cardiomyopathy. Am J Cardiol 57: 816–820PubMedCrossRefGoogle Scholar
- 20.Van der Bel-Kahn J (1977) Muscle fiber disarray in common heart disease. Am J Cardiol 40: 355–364PubMedCrossRefGoogle Scholar