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. 1995 Mar;73(3):284–289. doi: 10.1136/hrt.73.3.284

Endomyocardial fibrosis in Egypt: an illustrated review.

M A Rashwan 1, M Ayman 1, S Ashour 1, M M Hassanin 1, A A Zeina 1
PMCID: PMC483813  PMID: 7727191

Abstract

The detailed features of right sided endomyocardial fibrosis are described in 15 out of 10,000 consecutive patients who all had infection with Schistosoma mansoni and came from rural Egypt. Laboratory investigations, 12 lead electrocardiography, chest radiography, and Doppler echocardiography were performed in all patients. Cardiac catheterisation and angiography were performed in eight. Endomyocardial biopsy specimens were obtained from the right ventricles of two patients and pericardial biopsy specimens from two. Pericardiocentesis was performed in all patients. All patients were infected with S mansoni and had schistosomal hepatic fibrosis and ascites. Eleven had splenomegaly. All patients had raised cervical venous pressure with prominent Y descent and atrial fibrillation. Eosinophilia was notably absent. Echocardiography showed apical fibrosis in the right ventricle, obliteration of the ventricle, and moderate to massive exudative pericardial effusion in all patients. Calcification and fibrosis extended into the right ventricular outflow tracts in two patients. Huge right atrial thrombi occurred in five patients. Tricuspid regurgitation (grades I-II) was detected in 11 patients by Doppler ultrasonography. Haemodynamic and angiographic data confirmed the pure right sided restrictive pathophysiology. Pericardial biopsy specimens showed perivascular inflammatory infiltrates in two patients and a schistosomal granuloma in one. Endocardial biopsy specimens showed dense fibrosis with many fibroblasts. Endomyocardial fibrosis in Egypt is unique in several aspects. It always affected only the right side of the heart. Calcification and fibrosis extended to the right ventricular outflow tract. Pericardial inflammatory reaction was present. The relation to schistosomiasis and the link to periportal hepatic fibrosis in these patients is intriguing.

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Selected References

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  1. AL-ZAHAWI S., SHUKRI N. Histopathology of fatal myocarditis due to ectopic schistosomiasis. Trans R Soc Trop Med Hyg. 1956 Mar;50(2):166–168. doi: 10.1016/0035-9203(56)90078-5. [DOI] [PubMed] [Google Scholar]
  2. ANDRADE Z. A., GUIMARAES A. C. ENDOMYOCARDIAL FIBROSIS IN BAHIA, BRAZIL. Br Heart J. 1964 Nov;26:813–820. doi: 10.1136/hrt.26.6.813. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Abdel-Wahab M. F., Strickland G. T., El-Sahly A., El-Kady N., Zakaria S., Ahmed L. Changing pattern of schistosomiasis in Egypt 1935--79. Lancet. 1979 Aug 4;2(8136):242–244. doi: 10.1016/s0140-6736(79)90249-6. [DOI] [PubMed] [Google Scholar]
  4. Andrade Z. A., Andrade S. G. Pathogenesis of schistosomal pulmonary arteritis. Am J Trop Med Hyg. 1970 Mar;19(2):305–310. doi: 10.4269/ajtmh.1970.19.305. [DOI] [PubMed] [Google Scholar]
  5. BADAWI H., NOMEIR A. M. ELECTROCARDIOGRAMS OF RIGHT VENTRICULAR HYPERTROPHY IN BILHARZIAL COR PULMONALE. Br Heart J. 1965 May;27:355–364. doi: 10.1136/hrt.27.3.355. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. COCKSHOTT W. P. ANGIOCARDIOGRAPHY OF ENDOMYOCARDIAL FIBROSIS. Br J Radiol. 1965 Mar;38:192–200. doi: 10.1259/0007-1285-38-447-192. [DOI] [PubMed] [Google Scholar]
  7. Cheever A. W. A quantitative post-mortem study of Schistosomiasis mansoni in man. Am J Trop Med Hyg. 1968 Jan;17(1):38–64. doi: 10.4269/ajtmh.1968.17.38. [DOI] [PubMed] [Google Scholar]
  8. Cheever A. W., Kamel I. A., Elwi A. M., Mosimann J. E., Danner R., Sippel J. E. Schistosoma mansoni and S. haematobium infections in Egypt. III. Extrahepatic pathology. Am J Trop Med Hyg. 1978 Jan;27(1 Pt 1):55–75. doi: 10.4269/ajtmh.1978.27.55. [DOI] [PubMed] [Google Scholar]
  9. Cherian G., Vijayaraghavan G., Krishnaswami S., Sukumar I. P., John S., Jairaj P. S., Bhaktaviziam A. Endomyocardial fibrosis: report on the hemodynamic data in 29 patients and review of the results of surgery. Am Heart J. 1983 Apr;105(4):659–666. doi: 10.1016/0002-8703(83)90491-x. [DOI] [PubMed] [Google Scholar]
  10. Chopra P., Dave T. H. Right-sided endomyocardial fibrosis: an unusual morphological type. Int J Cardiol. 1990 Jun;27(3):383–385. doi: 10.1016/0167-5273(90)90297-i. [DOI] [PubMed] [Google Scholar]
  11. Chopra P., Narula J., Talwar K. K., Kumar V., Bhatia M. L. Histomorphologic characteristics of endomyocardial fibrosis: an endomyocardial biopsy study. Hum Pathol. 1990 Jun;21(6):613–616. doi: 10.1016/s0046-8177(96)90007-6. [DOI] [PubMed] [Google Scholar]
  12. Connor D. H., Somers K., Hutt M. S., Manion W. C., D'Arbela P. G. Endomyocardial fibrosis in Uganda (Davies' disease). 1. An epidemiologic, clinical, and pathologic study. Am Heart J. 1967 Nov;74(5):687–709. doi: 10.1016/0002-8703(67)90509-1. [DOI] [PubMed] [Google Scholar]
  13. Connor D. H., Somers K., Hutt M. S., Manion W. C., D'Arbela P. G. Endomyocardial fibrosis in Uganda (Davies' disease). II. An epidemiologic, clinical, and pathologic study. Am Heart J. 1968 Jan;75(1):107–124. doi: 10.1016/0002-8703(68)90122-1. [DOI] [PubMed] [Google Scholar]
  14. DAVIES J. N., BALL J. D. The pathology of endomyocardial fibrosis in Uganda. Br Heart J. 1955 Jul;17(3):337–359. doi: 10.1136/hrt.17.3.337. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Dunn M. A., Kamel R., Kamel I. A., Biempica L., Kholy A. E., Hait P. K., Rojkind M., Warren K. S., Mahmoud A. A. Liver collagen synthesis in schistosomiasis mansoni. Gastroenterology. 1979 May;76(5 Pt 1):978–982. [PubMed] [Google Scholar]
  16. Fawzy M. E., Ziady G., Halim M., Guindy R., Mercer E. N., Feteih N. Endomyocardial fibrosis: report of eight cases. J Am Coll Cardiol. 1985 Apr;5(4):983–988. doi: 10.1016/s0735-1097(85)80444-7. [DOI] [PubMed] [Google Scholar]
  17. Goodwin J. F. Endomyocardial disease--clinical features. Postgrad Med J. 1983 Mar;59(689):154–156. doi: 10.1136/pgmj.59.689.154. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Guimarães A. C., Santos Filho A., Esteves J. P., Vinhaes L. S., Abreu W. N. Hemodynamics in endomyocardial fibrosis. Am Heart J. 1974 Sep;88(3):294–303. doi: 10.1016/0002-8703(74)90462-1. [DOI] [PubMed] [Google Scholar]
  19. Homeida M., Abdel-Gadir A. F., Cheever A. W., Bennett J. L., Arbab B. M., Ibrahium S. Z., Abdel-Salam I. M., Dafalla A. A., Nash T. E. Diagnosis of pathologically confirmed Symmers' periportal fibrosis by ultrasonography: a prospective blinded study. Am J Trop Med Hyg. 1988 Jan;38(1):86–91. doi: 10.4269/ajtmh.1988.38.86. [DOI] [PubMed] [Google Scholar]
  20. Metras D., Coulibaly A. O., Ouattara K., Chauvet J., Ekra A., Longechaud A., Bertrand E. Endomyocardial fibrosis: early and late results of surgery in 20 patients. J Thorac Cardiovasc Surg. 1982 Jan;83(1):52–64. [PubMed] [Google Scholar]
  21. Moodie D. S., Baum J. E., Gill C. C., Ratliff N. B. Endomyocardial fibrosis. Diagnosis and surgical treatment of two cases occurring in the United States. Cleve Clin Q. 1986 Summer;53(2):159–170. doi: 10.3949/ccjm.53.2.159. [DOI] [PubMed] [Google Scholar]
  22. Olsen E. G., Spry C. J. Relation between eosinophilia and endomyocardial disease. Prog Cardiovasc Dis. 1985 Jan-Feb;27(4):241–254. doi: 10.1016/0033-0620(85)90008-8. [DOI] [PubMed] [Google Scholar]
  23. Roberts W. C., Liegler D. G., Carbone P. P. Endomyocardial disease and eosinophilia. A clinical and pathologic spectrum. Am J Med. 1969 Jan;46(1):28–42. doi: 10.1016/0002-9343(69)90055-2. [DOI] [PubMed] [Google Scholar]
  24. Shaper A. G., Kaplan M. H., Mody N. J., McIntyre P. A. Malarial antibodies and autoantibodies to heart and other tissues in the immigrant and indigenous peoples of Uganda. Lancet. 1968 Jun 22;1(7556):1342–1346. doi: 10.1016/s0140-6736(68)92037-0. [DOI] [PubMed] [Google Scholar]
  25. Singh N. P. The role of thorium in endomyocardial fibrosis. Hum Exp Toxicol. 1990 Mar;9(2):79–82. doi: 10.1177/096032719000900203. [DOI] [PubMed] [Google Scholar]
  26. Somers K., Brenton D. P., D'Arbela P. G., Fowler J. M., Kanyerezi B. R., Sood N. K. Haemodynamic features of severe endomyocardial fibrosis of right ventricle, including comparison with constrictive pericarditis. Br Heart J. 1968 May;30(3):322–332. doi: 10.1136/hrt.30.3.322. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Somers K., Brenton D. P., Sood N. K. Clinical features of endomyocardial fibrosis of the right ventricle. Br Heart J. 1968 May;30(3):309–321. doi: 10.1136/hrt.30.3.309. [DOI] [PMC free article] [PubMed] [Google Scholar]
  28. Somers K., Hutt M. S., Patel A. K., D'Arbela P. G. Endomyocardial biopsy in diagnosis of cardiomyopathies. Br Heart J. 1971 Nov;33(6):822–832. doi: 10.1136/hrt.33.6.822. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Stierle U., Tarbiat S., Nazarian I., Aryanpur I., Sheikhzadeh A. Right ventricular endomyocardial fibrosis. Diagnosis and management. Jpn Heart J. 1990 May;31(3):329–339. doi: 10.1536/ihj.31.329. [DOI] [PubMed] [Google Scholar]
  30. Valiathan M. S., Balakrishnan K. G., Kartha C. C. A profile of endomyocardial fibrosis. Indian J Pediatr. 1987 Mar-Apr;54(2):229–236. doi: 10.1007/BF02750815. [DOI] [PubMed] [Google Scholar]
  31. Valiathan M. S., Kartha C. C., Eapen J. T., Dang H. S., Sunta C. M. A geochemical basis for endomyocardial fibrosis. Cardiovasc Res. 1989 Jul;23(7):647–648. doi: 10.1093/cvr/23.7.647. [DOI] [PubMed] [Google Scholar]
  32. Vijayaraghavan G., Cherian G., Krishnaswami S., Sukumar I. P. Left ventricular endomyocardial fibrosis in India. Br Heart J. 1977 May;39(5):563–568. doi: 10.1136/hrt.39.5.563. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Vijayaraghavan G., Davies J., Sadanandan S., Spry C. J., Gibson D. G., Goodwin J. F. Echocardiographic features of tropical endomyocardial disease in South India. Br Heart J. 1983 Nov;50(5):450–459. doi: 10.1136/hrt.50.5.450. [DOI] [PMC free article] [PubMed] [Google Scholar]
  34. van der Horst R. Schistosomiasis of the pericardium. Trans R Soc Trop Med Hyg. 1979;73(2):243–244. doi: 10.1016/0035-9203(79)90227-x. [DOI] [PubMed] [Google Scholar]

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