Skip to main content
PMC home page
British Heart Journal logoLink to British Heart Journal
. 1980 Oct;44(4):433–443. doi: 10.1136/hrt.44.4.433

Histopathological specificity of hypertrophic obstructive cardiomyopathy. Myocardial fibre disarray and myocardial fibrosis.

M G St John Sutton, J T Lie, K R Anderson, P C O'Brien, R L Frye
PMCID: PMC482424  PMID: 7191711

Abstract

The topography and specificity of fibre disarray and fibrosis in hypertrophic obstructive cardiomyopathy were determined in a histological study comprising 40 necropsy hearts--10 with hypertrophic cardiomyopathy, 10 with congestive cardiomyopathy, 10 with aortic valve stenosis, and 10 normal hearts. Seven standard regional sections were sampled from each heart and graded "double-blind" (tissue location and disease entity) for severity and extent of fibre dissarray and four distinct types of myocardial fibrosis. Statistical comparison of the severity and distribution of indices of fibre disarray and fibrosis within each group and between the normal and the disease groups showed that fibre disarray and fibrosis were qualitatively non-specific for hypertrophic cardiomyopathy. However, when fibre disarray was quantified (1) it was significantly increased in hypertrophic cardiomyopathy and allowed separation of hearts with hypertrophic cardiomyopathy from normal hearts and from those with congestive cardiomyopathy and aortic stenosis, (2) it did not vary significantly among sections of the left ventricle (that is, between the septum and the free wall) in hypertrophic cardiomyopathy, (3) it was closely associated with plexiform fibrosis, and (4) it varied independently of wall and septal thickness. Though the histogenesis of fibre disarray is unknown, it probably represents an exaggeration of a non-specific common pathway for many diverse pathophysiological processes.

Full text

PDF
440

Images in this article

435Image
on p.435
436Image
on p.436

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Asymmetric septal hypertrophy. Ann Intern Med. 1974 Nov;81(5):650–680. doi: 10.7326/0003-4819-81-5-650. [DOI] [PubMed] [Google Scholar]
  2. BRAUNWALD E., LAMBREW C. T., ROCKOFF S. D., ROSS J., Jr, MORROW A. G. IDIOPATHIC HYPERTROPHIC SUBAORTIC STENOSIS. I. A DESCRIPTION OF THE DISEASE BASED UPON AN ANALYSIS OF 64 PATIENTS. Circulation. 1964 Nov;30:SUPPL 4–119. doi: 10.1161/01.cir.29.5s4.iv-3. [DOI] [PubMed] [Google Scholar]
  3. BROCK R. Functional obstruction of the left ventricle; acquired aortic subvalvar stenosis. Guys Hosp Rep. 1957;106(4):221–238. [PubMed] [Google Scholar]
  4. Bulkley B. H., Fortuin N. J. Systolic anterior motion of the mitral valve without asymmetric septal hypertrophy. Chest. 1976 May;69(5):694–696. doi: 10.1378/chest.69.5.694. [DOI] [PubMed] [Google Scholar]
  5. Bulkley B. H., Weisfeldt M. L., Hutchins G. M. Asymmetric septal hypertrophy and myocardial fiber disarray. Features of normal, developing, and malformed hearts. Circulation. 1977 Aug;56(2):292–298. doi: 10.1161/01.cir.56.2.292. [DOI] [PubMed] [Google Scholar]
  6. Bulkley B. H., Weisfeldt M. L., Hutchins G. M. Isometric cardiac contraction. a possible cause of the disorganized myocardial pattern of idiopathic hypertrophic subaortic stenosis. N Engl J Med. 1977 Jan 20;296(3):135–139. doi: 10.1056/NEJM197701202960303. [DOI] [PubMed] [Google Scholar]
  7. COHEN J., EFFAT H., GOODWIN J. F., OAKLEY C. M., STEINER R. E. HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY. Br Heart J. 1964 Jan;26:16–32. doi: 10.1136/hrt.26.1.16. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Clark C. E., Henry W. L., Epstein S. E. Familial prevalence and genetic transmission of idiopathic hypertrophic subaortic stenosis. N Engl J Med. 1973 Oct 4;289(14):709–714. doi: 10.1056/NEJM197310042891402. [DOI] [PubMed] [Google Scholar]
  9. Davies M. J., Pomerance A., Teare R. D. Pathological features of hypertrophic obstructive cardiomyopathy. J Clin Pathol. 1974 Jul;27(7):529–535. doi: 10.1136/jcp.27.7.529. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Ferrans V. J., Morrow A. G., Roberts W. C. Myocardial ultrastructure in idiopathic hypertrophic subaortic stenosis. A study of operatively excised left ventricular outflow tract muscle in 14 patients. Circulation. 1972 Apr;45(4):769–792. doi: 10.1161/01.cir.45.4.769. [DOI] [PubMed] [Google Scholar]
  11. GOODWIN J. F., HOLLMAN A., CLELAND W. P., TEARE D. Obstructive cardiomyopathy simulating aortic stenosis. Br Heart J. 1960 Jun;22:403–414. doi: 10.1136/hrt.22.3.403. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Henry W. L., Clark C. E., Epstein S. E. Asymmetric septal hypertrophy (ASH): the unifying link in the IHSS disease spectrum. Observations regarding its pathogenesis, pathophysiology, and course. Circulation. 1973 Apr;47(4):827–832. doi: 10.1161/01.cir.47.4.827. [DOI] [PubMed] [Google Scholar]
  13. Henry W. L., Clark C. E., Epstein S. E. Asymmetric septal hypertrophy. Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation. 1973 Feb;47(2):225–233. doi: 10.1161/01.cir.47.2.225. [DOI] [PubMed] [Google Scholar]
  14. James T. N., Marshall T. K. De subitaneis mortibus. XII. Asymmetrical hypertrophy of the heart. Circulation. 1975 Jun;51(6):1149–1166. doi: 10.1161/01.cir.51.6.1149. [DOI] [PubMed] [Google Scholar]
  15. Jones M., Ferrans V. J., Morrow A. G., Roberts W. C. Ultrastructure of crista supraventricularis muscle in patients with congenital heart diseases associated with right ventricular outflow tract obstruction. Circulation. 1975 Jan;51(1):39–67. doi: 10.1161/01.cir.51.1.39. [DOI] [PubMed] [Google Scholar]
  16. Larter W. E., Allen H. D., Sahn D. J., Goldberg S. J. The asymmetrically hypertrophied septum. Further differentiation of its causes. Circulation. 1976 Jan;53(1):19–27. doi: 10.1161/01.cir.53.1.19. [DOI] [PubMed] [Google Scholar]
  17. Manasek F. J. Histogenesis of the embryonic myocardium. Am J Cardiol. 1970 Feb;25(2):149–168. doi: 10.1016/0002-9149(70)90576-x. [DOI] [PubMed] [Google Scholar]
  18. Maron B. J., Clark C. E., Henry W. L., Fukuda T., Edwards J. E., Mathews E. C., Jr, Redwood D. R., Epstein S. E. Prevalence and characteristics of disproportionate ventricular septal thickening in patients with acquired or congenital heart diseases: echocardiographic and morphologic findings. Circulation. 1977 Mar;55(3):489–496. doi: 10.1161/01.cir.55.3.489. [DOI] [PubMed] [Google Scholar]
  19. Maron B. J., Edwards J. E., Ferrans V. J., Clark C. E., Lebowitz E. A., Henry W. L., Epstein S. E. Congenital heart malformations associated with disproportionate ventricular septal thickening. Circulation. 1975 Nov;52(5):926–932. doi: 10.1161/01.cir.52.5.926. [DOI] [PubMed] [Google Scholar]
  20. Maron B. J., Edwards J. E., Henry W. L., Clark C. E., Bingle G. J., Epstein S. E. Asymmetric septal hypertrophy (ASH) in infancy. Circulation. 1974 Oct;50(4):809–820. doi: 10.1161/01.cir.50.4.809. [DOI] [PubMed] [Google Scholar]
  21. Maron B. J., Ferrans V. J., Henry W. L., Clark C. E., Redwood D. R., Roberts W. C., Morrow A. G., Epstein S. E. Differences in distribution of myocardial abnormalities in patients with obstructive and nonobstructive asymmetric septal hypertrophy (ASH). Light and electron microscopic findings. Circulation. 1974 Sep;50(3):436–446. doi: 10.1161/01.cir.50.3.436. [DOI] [PubMed] [Google Scholar]
  22. Maron B. J., Ferrans V. J., White R. I., Jr Unusual evolution of acquired infundibular stenosis in patients with ventricular septal defect. Clinical and morphologic observations. Circulation. 1973 Nov;48(5):1092–1103. doi: 10.1161/01.cir.48.5.1092. [DOI] [PubMed] [Google Scholar]
  23. Roberts W. C., Ferrans V. J. Pathologic anatomy of the cardiomyopathies. Idiopathic dilated and hypertrophic types, infiltrative types, and endomyocardial disease with and without eosinophilia. Hum Pathol. 1975 May;6(3):287–342. [PubMed] [Google Scholar]
  24. SHABETAI R., McGUIRE J. Idiopathic cardiac hypertrophy simulating valvular heart disease. Am Heart J. 1963 Jan;65:124–130. doi: 10.1016/0002-8703(63)90222-9. [DOI] [PubMed] [Google Scholar]
  25. SOULIE P., JOLY F., CARLOTTI J. [Idiopathic stenoses of the outflow chamber of the left ventricle. (Apropos of 10 cases)]. Acta Cardiol. 1962;17:335–378. [PubMed] [Google Scholar]
  26. Sanderson J. E., Gibson D. G., Brown D. J., Goodwin J. F. Left ventricular filling in hypertrophic cardiomyopathy. An angiographic study. Br Heart J. 1977 Jun;39(6):661–670. doi: 10.1136/hrt.39.6.661. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Sanderson J. E., Traill T. A., Sutton M. G., Brown D. J., Gibson D. G., Goodwin J. F. Left ventricular relaxation and filling in hypertrophic cardiomyopathy. An echocardiographic study. Br Heart J. 1978 Jun;40(6):596–601. doi: 10.1136/hrt.40.6.596. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Shah P. M., Gramiak R., Adelman A. G., Wigle E. D. Role of echocardiography in diagnostic and hemodynamic assessment of hypertrophic subaortic stenosis. Circulation. 1971 Nov;44(5):891–898. doi: 10.1161/01.cir.44.5.891. [DOI] [PubMed] [Google Scholar]
  29. Sutton M. G., Tajik A. J., Gibson D. G., Brown D. J., Seward J. B., Guiliani E. R. Echocardiographic assessment of left ventricular filling and septal and posterior wall dynamics in idiopathic hypertrophic subaortic stenosis. Circulation. 1978 Mar;57(3):512–520. doi: 10.1161/01.cir.57.3.512. [DOI] [PubMed] [Google Scholar]
  30. TEARE D. Asymmetrical hypertrophy of the heart in young adults. Br Heart J. 1958 Jan;20(1):1–8. doi: 10.1136/hrt.20.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Van Noorden S., Olsen E. G., Pearse A. G. Hypertrophic obstructive cardiomyopathy, a histological, histochemical, and ultrastructural study of biopsy material. Cardiovasc Res. 1971 Jan;5(1):118–131. doi: 10.1093/cvr/5.1.118. [DOI] [PubMed] [Google Scholar]
  32. van der Bel-Kahn J. Muscle fiber disarray in common heart diseases. Am J Cardiol. 1977 Sep;40(3):355–364. doi: 10.1016/0002-9149(77)90157-6. [DOI] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES