Skip to main content
PMC home page
British Heart Journal logoLink to British Heart Journal
. 1982 Jun;47(6):527–538. doi: 10.1136/hrt.47.6.527

Myocardial disarray. A critical review.

A E Becker, G Caruso
PMCID: PMC481178  PMID: 7044398

Abstract

Myocardial disarray or disorganisation is at present a contentious topic, not least because its value as a clinical marker for hypertrophic cardiomyopathy has changed considerably over the years. Initially observed as one of the features of asymmetric septal hypertrophy, disarray has since been promoted as its pathognomonic histological feature, regarded by some observers as the morphological manifestation of a genetically transmitted myocardial defect. Recently, however, it has become evident that myocardial disarray is not limited to hypertrophic cardiomyopathy, but is encountered in hearts with both congenital and acquired conditions, and is also observed in normal hearts. The specificity of disarray for hypertrophic cardiomyopathy is thus seriously questioned. Latterly, it has been suggested that disarray, judged from through-and-through sections of the ventricular midseptum is a highly specific and sensitive marker of hypertrophic cardiomyopathy when considered in quantitative rather than qualitative fashion. The present study sets out to answer the question whether disarray could be the histological expression of the normal but intricate fibre architecture of the heart, a consideration also initiated by debatable definitions of normality and abnormality of myocardial histology. Gross fibre dissections in five normal hearts showed that many sites occurred in which disarray was a natural phenomenon. In five more hearts it was found that the plane of section of a tissue block might profoundly influence the histology. In fact, tissue cubicles sampled from different faces showed a change in histology in the vast majority. Thus the diagnostic significance of myocardial disarray as a marker of hypertrophic cardiomyopathy in the clinical setting almost vanishes; a change in orientation of a tissue section may actually turn "normality" into "disarray".

Full text

PDF
527

Images in this article

530Image
on p.530
531Image
on p.531
532Image
on p.532
533Image
on p.533
534Image
on p.534
535Image
on p.535
536Image
on p.536

Selected References

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

  1. Beçu L., Somerville J., Gallo A. 'Isolated' pulmonary valve stenosis as part of more widespread cardiovascular disease. Br Heart J. 1976 May;38(5):472–482. doi: 10.1136/hrt.38.5.472. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Bulkley B. H. Idiopathic hypertrophic subaortic stenosis afflicted: idols of the cave and the marketplace. Am J Cardiol. 1977 Sep;40(3):476–479. doi: 10.1016/0002-9149(77)90176-x. [DOI] [PubMed] [Google Scholar]
  3. 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]
  4. 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]
  5. 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]
  6. Edwards W. D., Zakheim R., Mattioli L. Asymmetric septal hypertrophy in childhood. Unreliability of histologic criteria for differentiation of obstructive and nonobstructive forms. Hum Pathol. 1977 May;8(3):277–284. doi: 10.1016/s0046-8177(77)80024-5. [DOI] [PubMed] [Google Scholar]
  7. 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]
  8. Ferrans V. J., Roberts W. C. Myocardial biopsy: a useful diagnostic procedure or only a research tool? Am J Cardiol. 1978 May 1;41(5):965–967. doi: 10.1016/0002-9149(78)90741-5. [DOI] [PubMed] [Google Scholar]
  9. Fox C. C., Hutchins G. M. The architecture of the human ventricular myocardium. Johns Hopkins Med J. 1972 May;130(5):289–299. [PubMed] [Google Scholar]
  10. Fujita M., Neustein H. B., Lurie P. R. Transvascular endomyocardial biopsy in infants and small children. Myocardial findings in 10 cases of cardiomyopathy. Hum Pathol. 1979 Jan;10(1):15–30. doi: 10.1016/s0046-8177(79)80069-6. [DOI] [PubMed] [Google Scholar]
  11. Fujiwara H., Kawai C., Hamashima Y. Myocardial fascicle and fiber disarray in 25 mu-thick sections. Circulation. 1979 Jun;59(6):1293–1298. doi: 10.1161/01.cir.59.6.1293. [DOI] [PubMed] [Google Scholar]
  12. GRANT R. P. NOTES ON THE MUSCULAR ARCHITECTURE OF THE LEFT VENTRICLE. Circulation. 1965 Aug;32:301–308. doi: 10.1161/01.cir.32.2.301. [DOI] [PubMed] [Google Scholar]
  13. Henry W. L., Clark C. E., 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). Echocardiographic and gross anatomic findings. Circulation. 1974 Sep;50(3):447–455. doi: 10.1161/01.cir.50.3.447. [DOI] [PubMed] [Google Scholar]
  14. Hutchins G. M., Bulkley B. H. Catenoid shape of the interventricular septum: possible cause of idiopathic hypertrophic subaortic stenosis. Circulation. 1978 Sep;58(3 Pt 1):392–397. doi: 10.1161/01.cir.58.3.392. [DOI] [PubMed] [Google Scholar]
  15. Isner J. M., Maron B. J., Roberts W. C. Comparison of amount of myocardial cell disorganization in operatively excised septectomy specimens with amount observed at necropsy in 18 patients with hypertrophic cardiomyopathy. Am J Cardiol. 1980 Jul;46(1):42–47. doi: 10.1016/0002-9149(80)90603-7. [DOI] [PubMed] [Google Scholar]
  16. 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]
  17. LEV M., SIMKINS C. S. Architecture of the human ventricular myocardium; technic for study using a modification of the Mall-MacCallum method. Lab Invest. 1956 Sep-Oct;5(5):396–409. [PubMed] [Google Scholar]
  18. Liu S. K., Maron B. J., Tilley L. P. Feline hypertrophic cardiomyopathy: gross anatomic and quantitative histologic features. Am J Pathol. 1981 Mar;102(3):388–395. [PMC free article] [PubMed] [Google Scholar]
  19. Liu S. K., Maron B. J., Tilley L. P. Hypertrophic cardiomyopathy in the dog. Am J Pathol. 1979 Mar;94(3):497–508. [PMC free article] [PubMed] [Google Scholar]
  20. Maron B. J., Anan T. J., Roberts W. C. Quantitative analysis of the distribution of cardiac muscle cell disorganization in the left ventricular wall of patients with hypertrophic cardiomyopathy. Circulation. 1981 Apr;63(4):882–894. doi: 10.1161/01.cir.63.4.882. [DOI] [PubMed] [Google Scholar]
  21. 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]
  22. Maron B. J., Edwards J. E., Moller J. H., Epstein S. E. Prevalence and characteristics of disporportionate ventricular septal thickening in infants with congenital heart disease. Circulation. 1979 Jan;59(1):126–133. doi: 10.1161/01.cir.59.1.126. [DOI] [PubMed] [Google Scholar]
  23. Maron B. J., Epstein S. E. Hypertrophic cardiomyopathy. Recent observations regarding the specificity of three hallmarks of the disease: asymmetric septal hypertrophy, septal disorganization and systolic anterior motion of the anterior mitral leaflet. Am J Cardiol. 1980 Jan;45(1):141–154. doi: 10.1016/0002-9149(80)90232-5. [DOI] [PubMed] [Google Scholar]
  24. 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]
  25. Maron B. J., Henry W. L., Clark C. E., Redwood D. R., Roberts W. C., Epstein S. E. Asymetric septal hypertrophy in childhood. Circulation. 1976 Jan;53(1):9–19. doi: 10.1161/01.cir.53.1.9. [DOI] [PubMed] [Google Scholar]
  26. Maron B. J., Roberts W. C. Hypertrophic cardiomyopathy and cardiac muscle cell disorganization revisited: relation between the two and significance. Am Heart J. 1981 Jul;102(1):95–110. doi: 10.1016/0002-8703(81)90419-1. [DOI] [PubMed] [Google Scholar]
  27. Maron B. J., Roberts W. C. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophic cardiomyopathy. Circulation. 1979 Apr;59(4):689–706. doi: 10.1161/01.cir.59.4.689. [DOI] [PubMed] [Google Scholar]
  28. Maron B. J., Sato N., Roberts W. C., Edwards J. E., Chandra R. S. Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum. Comparison of fetuses and infants with and without congenital heart disease and patients with hypertrophic cardiomyopathy. Circulation. 1979 Sep;60(3):685–696. doi: 10.1161/01.cir.60.3.685. [DOI] [PubMed] [Google Scholar]
  29. Millhouse E. W., Jr, Chiakulas J. J., Scheving L. E. Long-term organ culture of the salamander heart. J Cell Biol. 1971 Jan;48(1):1–14. doi: 10.1083/jcb.48.1.1. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Oakley C. M. Clinical definitions and classification of cardiomyopathies. Postgrad Med J. 1972 Dec;48(566):703–713. doi: 10.1136/pgmj.48.566.703. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. PARE J. A., FRASER R. G., PIROZYNSKI W. J., SHANKS J. A., STUBINGTON D. Hereditary cardiovascular dysplasia. A form of familial cardiomyopathy. Am J Med. 1961 Jul;31:37–62. doi: 10.1016/0002-9343(61)90222-4. [DOI] [PubMed] [Google Scholar]
  32. Perloff J. K. Pathogenesis of hypertrophic cardiomyopathy: hypotheses and speculations. Am Heart J. 1981 Feb;101(2):219–226. doi: 10.1016/0002-8703(81)90669-4. [DOI] [PubMed] [Google Scholar]
  33. Ross A. A., Streeter D. D., Jr Myocardial fiber disarray. Circulation. 1979 Dec;60(6):1425–1426. [PubMed] [Google Scholar]
  34. STAMPBACH O., SENN A. [Idiopathic hypertrophic subaortic stenosis]. Schweiz Med Wochenschr. 1962 Feb 3;92:125–130. [PubMed] [Google Scholar]
  35. Snijder J., de Jong J., Meijer A. E. Histopathological, enzyme-histochemical and electron-microscopical observations in hypertrophic obstructive cardiomyopathy. A preliminary report. Pathol Microbiol (Basel) 1970;35(1):81–85. doi: 10.1159/000162203. [DOI] [PubMed] [Google Scholar]
  36. St John Sutton M. G., Lie J. T., Anderson K. R., O'Brien P. C., Frye R. L. Histopathological specificity of hypertrophic obstructive cardiomyopathy. Myocardial fibre disarray and myocardial fibrosis. Br Heart J. 1980 Oct;44(4):433–443. doi: 10.1136/hrt.44.4.433. [DOI] [PMC free article] [PubMed] [Google Scholar]
  37. 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]
  38. 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]
  39. Wigle E. D., Silver M. D. Myocardial fiber disarray and ventricular septal hypertrophy in asymmetrical hypertrophy of the heart. Circulation. 1978 Sep;58(3 Pt 1):398–402. doi: 10.1161/01.cir.58.3.398. [DOI] [PubMed] [Google Scholar]
  40. 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