Skip to main content
SpringerLink
Log in

Favorable effects of immunosuppressive therapy in children with dilated cardiomyopathy and active myocarditis

  • Original Articles
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Among 68 children with severe dilated cardiomyopathy, 43 (aged 10 months to 15 years) presented with active myocarditis, diagnosed by endomyocardial biopsy. They were divided into four treatment groups: I, controls: 9 patients submitted to conventional treatment (digitalis, diuretics, and vasodilators) for 8.1±0.7 (SD) months; II, prednisone: 12 patients received conventional therapy plus prednisone; III, azathioprine: 16 patients submitted to conventional therapy plus prednisone and azathioprine; IV, cyclosporine: 13 patients treated with conventional therapy plus prednisone and cyclosporine. Immunosuppressive therapy was maintained for a mean of 8.4±1.2 months. They were submitted to noninvasive (electrocardiogram, chest radiograph, Doppler echocardiogram, and radioisotopic scintigraphy) and invasive (hemodynamic) studies. In the control group only 2 of 9 patients showed clinical and hemodynamic improvement and 1 of 4, histologic regression of the myocarditis. Among patients submitted to conventional therapy plus prednisone, 3 of 12 presented clinical and hemodynamic improvement; 2 of 5 also showed histologic regression of inflammatory process. By contrast, patients treated with azathioprine or cyclosporine associated with prednisone had significantly better results: 13 of 16 and 10 of 13 patients, respectively, had clinical and hemodynamic improvement; all 6 patients in the azathioprine group and all 4 patients in the cyclosporine group had histologic regression of the myocarditis. Two patients in the prednisone group, one in the azathioprine group, and one in the cyclosporine group died during treatment, in cardiogenic shock. In our experience immunosuppressive therapy with azathioprine or cyclosporine associated with prednisone improves the prognosis of children with active myocarditis and severe ventricular dysfunction.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Ainger LE (1964) Acute aseptic myocarditis: corticosteroid therapy.J Pediatr 64:716–723

    Google Scholar 

  2. Baandrup O, Florio RA, Rehahn M, Richardson PJ, Olsen EGJ (1981) Critical analysis of endomyocardial biopsies from patients suspected of having cardiomyopathy. II. Comparison of histology and clinical/haemodynamic information.Br Heart J 45:487–493

    Google Scholar 

  3. Baandrup O, Olsen EGJ (1981) Critical analysis of endomyocardial biopsies from patients suspected of having cardiomyopathy. I. Morphological and morphometric aspects.Br Heart J 45:475–486

    Google Scholar 

  4. Camargo PR, Mazzieri R, Snitcowsky R, et al (1990) Correlation between gallium-67 imaging and endomyocardial biopsy.Int J Cardiol 28:293–298

    Google Scholar 

  5. Cassling RS, Linder J, Sears TD, et al (1985) Quantitative evaluation of inflammation in biopsy specimens from idiopathically failing or irritable hearts: experience in 80 pediatric and adult patients.Am Heart J 110:713–720

    Google Scholar 

  6. Caves PK, Stinson EB, Graham AF, et al (1973) Percutaneous transvenous endomyocardial biopsy.JAMA 225:288–291

    Google Scholar 

  7. Chan KY, Iwahara M, Benson LN, Wilson GJ, Freedom RM (1991) Immunosuppressive therapy in the management of acute myocarditis in children: a clinical trial.J Am Coll Cardiol 17:458–460

    Google Scholar 

  8. Daly K, Richardson PJ, Olsen EGJ, et al (1984) Role of histologic and virological examination in the diagnosis and assessment of immunosuppressive treatment.Br Heart J 51: 30–35

    Google Scholar 

  9. Dec GW Jr, Fallon JT, Southern JF, Palacios IF (1988) Relation between histologic findings on early repeated right ventricular biopsy and ventricular function in patients with myocarditis.Br Heart J 60:332–337

    Google Scholar 

  10. Dec GW, Fallon JT, Southern JF, Palacios I (1990) “Borderline” myocarditis: an indication for repeat endomyocardial biopsy.J Am Col Cardiol 15:283–289

    Google Scholar 

  11. Dec GW, Palacios TF, Pallon JT, et al (1985) Active myocarditis in the spectrum of acute dilated cardiomyopathies: clinical features, histologic correlates, and clinical outcome.N Engl J Med 312:885–890

    Google Scholar 

  12. Fenoglio JJ Jr, Ursell PC, Kellog CF, Drusin RE, Weiss MB (1983) Diagnosis and classification of myocarditis by endomyocardial biopsy.N Engl J Med 301:12–18

    Google Scholar 

  13. Fowles RE, Mason JW (1982) Endomyocardial biopsy.Ann Intern Med 97:885–894

    Google Scholar 

  14. French WJ, Criley JM (1984) Caution in the diagnosis and treatment of myocarditis.Am J Cardiol 54:445–446

    Google Scholar 

  15. Higuchi ML, Morais CF, Pereira-Barretto AC, et al (1987) The role of active myocarditis in the development of heart failure in chronic Chagas' disease: a study based on endomyocardial biopsies.Clin Cardiol 10:665–670

    Google Scholar 

  16. Higuchi ML, Morais CF, Sambiase NV, et al (1990) Histopathologic criteria of myocarditis: a study based on normal heart, chagasic and dilated cardiomyopathy.Jpn Circ J 54: 391–400

    Google Scholar 

  17. Hosenpud JD, McAnulty JH, Niles NR (1985) Lack of objective improvement in ventricular systolic function in patients with myocarditis treated with azathioprine and prednisone.J Am Coll Cardiol 6:797–801

    Google Scholar 

  18. Kawamura K, Kitaura Y, Morita H, Deguchi H, Kotaka M (1985) Viral and idiopathic myocarditis in Japan: a questionnaire survey. In: Sekiguchi M, Olsen EGJ, Goodwin JF (eds)Myocarditis and Related Disorders. Springer-Verlag. Tokyo, 1985, pp. 18–22

    Google Scholar 

  19. Lie JT (1988) Myocarditis and endomyocardial biopsy in unexplained heart failure: a diagnosis in search of a disease.Ann Intern Med 109:525–528.

    Google Scholar 

  20. Lieberman EB, Hutchins GM, Herskowitz A, Rose NR, Baughman KL (1991) Clinicopathologic description of myocarditis.J Am Coll Cardiol 18:1617–1626

    Google Scholar 

  21. Mason JW, Billingham ME, Ricci DR (1980) Treatment of acute inflammatory myocarditis assisted by endomyocardial biopsy.Am J Cardiol 45:1037–1044

    Google Scholar 

  22. Mortensen SA, Basandrup V, Bush J, Bentsenk K, Hvid-Jacobsen K (1985) Immunosuppressive therapy of biopsy-proven myocarditis: experiences with corticosteroid and cycloporin.Int J Immunother 1:34–45

    Google Scholar 

  23. Nippoldt TB, Edwards WD, Holmes DR Jr, et al (1982) Right ventricular endomyocardial biopsy: clinicopathologic correlates in 100 consecutive patients.Mayo Clin Proc 47: 407–418

    Google Scholar 

  24. O'Connell JB, Henkin RE, Robinson JA, et al (1984) Gallium-67 imaging in patients with dilated cardiomyopathy and biopsy proven myocarditis.Circulation 70:58–62

    Google Scholar 

  25. O'Connell JB, Robinson JA, Henkin RE, Gunnar RM (1981) Immunosuppressive therapy in patients with congestive cardiomyopathy and myocardial uptake of gallium-67.Circulation 64:780–786

    Google Scholar 

  26. Okuni M, Yamada T, Mochizuki S, Sakurai I (1975) Studies on myocarditis in childhood with special reference to the possible role of immunological process and the thymus in the chronicity of the disease.Jpn Circ J 39:463–470

    Google Scholar 

  27. Olsen EGJ (1985) The role of biopsy in the diagnosis of myocarditis.Herz 10:21–26

    Google Scholar 

  28. Parillo JE, Aretz HT, Palacios I, Fallon JT, Block PC (1984) The results of transvenous endomyocardial biopsy can frequently be used to diagnose myocardial diseases in patients with idiopathic heart failure.Circulation 69:93–101

    Google Scholar 

  29. Richardson PJ (1985) Immunosuppressive therapy in the management of biopsy proven acute myocarditis.Herz 10: 36–43

    Google Scholar 

  30. Salvi A, Di Lenarda A, Dreas L, Silvestri F, Camerini F (1989) Immunosuppressive treatment in myocarditis.Int J Cardiol 22:329–338

    Google Scholar 

  31. Sekiguchi M, Hiroe M, Take M, Hirosawa K (1980) Clinical and histopathological profile of sarcoidosis of the heart and acute idiopathic myocarditis: concepts through a study employing endomyocardial biopsy. II. Myocarditis.Jpn Circ J 44:264–273

    Google Scholar 

  32. Sugrue DD, Holmes DR Jr, Gersh BJ, et al (1984) Cardiac histologic findings in patients with life-threatening ventricular arrhythmias of unknown origin.J Am Coll Cardiol 5:952–1057

    Google Scholar 

  33. Woodruff JF (1980) Viral myocarditis: a review.Am J Pathol 101:427–484

    Google Scholar 

  34. Yang SS, Bentivoglio LG, Maranhåo V, Goldberg H (1972)From Cardiac Catheterization Data to Hemodynamic Parameters. Davis, Philadelphia, 1972

    Google Scholar 

  35. Zee-Cheng CS, Tsai CC, Palmer DC, et al (1984) High incidence of myocarditis by endomyocardial biopsy in patients with idiopathic congestive cardiomyopathy.J Am Coll Cardiol 3:63–70

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Divisao Clínica, Instituto do Coraçao, Av. Dr. Enéas Carvalho Aguiar 44, SP 05403-000, Sao Paulo, Brazil

    P. R. Camargo, R. Snitcowsky, P. L. da Luz, R. Mazzieri, M. L. Higuchi, M. Rati, N. Stolf, M. Ebaid & F. Pileggi

Authors
  1. P. R. Camargo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. R. Snitcowsky
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. P. L. da Luz
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R. Mazzieri
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. M. L. Higuchi
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. Rati
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. N. Stolf
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. M. Ebaid
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. F. Pileggi
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Camargo, P.R., Snitcowsky, R., da Luz, P.L. et al. Favorable effects of immunosuppressive therapy in children with dilated cardiomyopathy and active myocarditis. Pediatr Cardiol 16, 61–68 (1995). https://doi.org/10.1007/BF00796819

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00796819

Key Words

Search

Navigation