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Small Steps for Idiopathic Giant Cell Myocarditis

  • Management of Heart Failure (T Meyer, Section Editor)
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Abstract

Idiopathic giant cell myocarditis (IGCM) is a rare disease causing progressive myocarditis characterized by myocardial necrosis and giant cells. Patients often present with rapidly progressive heart failure, ventricular arrhythmias, and heart block. Without treatment, the disease often results in progressive pump failure requiring urgent cardiac transplantation or the need for mechanical circulatory support. The underlying pathophysiologic mechanisms are not yet defined but appear to involve genetics, autoimmune disorders, and possibly environmental factors such as viruses. Combined immunosuppressive regimens appear to prolong survival from death or cardiac transplant. Nevertheless, cardiac transplant is an effective treatment. The disease can recur in the transplanted heart resulting in death or the need for retransplant.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Saltykow S. Uber diffuse myokarditis. Virchows Arch Pathol Anat. 1905;182:1–39.

    Article  Google Scholar 

  2. Cooper L, Berry G, Shabetai R. Idiopathic giant-cell myocarditis—natural history and treatment. NEJM. 1997;336:1860–6. First, large, multicenter registry on the natural history, outcomes, and treatment of IGCM.

    Article  PubMed  Google Scholar 

  3. Kandolin R, Lehtonen J, Salmenkivi K, et al. Diagnosis, treatment, and outcome of giant-cell myocarditis in the era of combined immunosuppression. Circ: Heart Fail. 2013;6(1):15–22. Most recent, large registry on outcomes and treatment of IGCM.

    CAS  Google Scholar 

  4. Whitehead R. Isolated myocarditis. Br Heart J. 1965;27:220–30.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  5. Okada R, Wakafuji S. Myocarditis in autopsy. Heart Vessels Suppl. 1985;1:23–9.

    Article  CAS  PubMed  Google Scholar 

  6. Vaideeswar P, Cooper L. Giant cell myocarditis: clinical and pathological disease characteristics in an Indian population. Cardiovasc Pathol. 2013;22:70–4.

    Article  PubMed  Google Scholar 

  7. Drut RM. Giant-cell myocarditis in a newborn with congenital herpes simplex virus infection: an immunohistochemical study on the origin of the giant cells. Pediatr Pathol. 1986;6:431–7.

    Article  CAS  PubMed  Google Scholar 

  8. Meyer T, Grumbach IM, Kreuzer H, et al. Giant cell myocarditis due to coxsackie B2 virus infection. Cardiology. 1997;88:296–9.

    Article  CAS  PubMed  Google Scholar 

  9. Dennert R, Schalla S, Suylen RJ, et al. Giant cell myocarditis triggered by a parvovirus B19 infection. Int J Cardiol. 2009;134:115–6.

    Article  CAS  PubMed  Google Scholar 

  10. Everett RJ, Sheppard MN, Lefroy DC. Chest pain and palpitations: taking a closer look. Circulation. 2013;128(3):271–7.

    Article  PubMed  Google Scholar 

  11. Shariff S, Straatiamn L, Allard M, et al. Novel associations of giant cell myocarditis: tow case reports and a review of the literature. Can J Cardiol. 2004;4(5):557–61.

    Google Scholar 

  12. Burke JS, Medline NM, Kratz A. Giant cell myocarditis and myositis: associated with thymoma and myastenia gravis. Arch Pathol. 1969;88:359–66.

    CAS  PubMed  Google Scholar 

  13. Schumann C, Faust M, Gerharz M, et al. Autoimmune polyglandular syndrome associated with idiopathic giant cell myocarditis. Exp Clin Endocrinol Diabetes. 2005;113:302–7.

    Article  CAS  PubMed  Google Scholar 

  14. Mascaro-Blanco A, Alevarez K, Yu X, et al. Consequences of unlocking the cardiac myosin molecule in human myocarditis and cardiomyopathies. Autoimmunity. 2008;41:442–53.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  15. Okura Y, Takeda K, Honda S, et al. Recombinant murine interleukin-12 facilitates induction of cardiac myosin-specific type 1 helper T cells in rats. Circ Res. 1998;82:1035–42.

    Article  CAS  PubMed  Google Scholar 

  16. Gries W, Farkas D, Winters G, et al. Giant cell myocarditis: first report of disease recurrent in the transplanted heart. J Heart Lung Transplant. 1992;11:370–4.

    CAS  PubMed  Google Scholar 

  17. Kong G, Madden B, Spyrou N, et al. Response of recurrent giant cell myocarditis in a transplanted heart to intensive immunosuppression. Eur Heart J. 1991;12:554–7.

    CAS  PubMed  Google Scholar 

  18. Elezkurtaj S, Lassner D, Schultheiss H, et al. Vascular involvement in cardiac giant cell myocarditis: a new pathophysiological aspect. Clin Res Cardiol. 2014;103:161–3.

    Article  CAS  PubMed  Google Scholar 

  19. Kittleson M, Minhas K, Irizarry R, et al. Gene expression in giant cell myocarditis: altered expression of immune response genes. Int J Cardiol. 2005;102:333–40.

    Article  PubMed  Google Scholar 

  20. Shioji K, Kishimoto C, Nakayama Y, et al. Strain differences in rats with experimental giant cell myocarditis. Jpn Circ J. 2000;64:283–6.

    Article  CAS  PubMed  Google Scholar 

  21. Asimaki A, Tandri H, Duffy E, et al. Altered desmosomal proteins in granulomatous myocarditis and potential pathogenic links to arrhythmogenic right ventricular cardiomyopathy. Circ Arrhythm Electrophysiol. 2011;4:743–52.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  22. Fairweather D, Peri M, Coronado M, Cooper L. Autoimmune heart disease: role of sex hormones and autoantibodies in disease pathogenesis. Expert Rev Clin Immunol. 2012;8(3):269–84.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  23. Larsen BT, Maleszewski JJ, Edwards WD, Cooper LT, Sobonya RE, Thompson VE, et al. Atrial giant cell myocarditis: a distinctive clinicopathologic entity. Circulation. 2013;127:39–47.

    Article  PubMed  Google Scholar 

  24. Husband EM, Lannigan R. Unusual giant cell lesions in biopsy specimen of left atrial appendages in mitral stenosis. Br Heart J. 1965;27:269–73.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Gillie I, Fox H. Mitral stenosis together with a giant cell myocarditis limited to the left atrium. J Clin Pathol. 1968;21:750–2.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  26. Ahmad I, Craig MD, Berry GJ, Hsia HH, Wang PJ, Al-Ahmad A. Isolated giant cell myocarditis in the atrium: an incidental finding? Pacing Clin Electrophysiol. 2006;29:1179–80.

    Article  PubMed  Google Scholar 

  27. Bose AK, Bhattacharjee M, Martin V, Kendall S. Giant cell myocarditis of the left atrium. Cardiovasc Pathol. 2010;19:e37–8.

    Article  PubMed  Google Scholar 

  28. Shields RC, Tazelaar HD, Berry GJ, et al. The role of right ventricular endomyocardial biopsy for idiopathic giant cell myocarditis. J Card Fail. 2002;8:74–78c.

    Article  PubMed  Google Scholar 

  29. Sujino Y, Kimura F, Tanno J. Cardiac magnetic resonance imaging in giant cell myocarditis: intriguing associations with clinical and pathological features. Circulation. 2014;129(17):e467–9.

    Article  PubMed  Google Scholar 

  30. Litovsky SH, Burke AP, Virmani R. Giant cell myocarditis: an entity distinct from sarcoidosis characterized by multiphasic myocyte destruction by cytotoxic T cells and histiocytic giant cells. Mod Pathol. 1996;9(12):1126.

    CAS  PubMed  Google Scholar 

  31. Davies MJ, Pomerance A, Teare RD. Idiopathic giant cell myocarditis—a distinctive clinico-pathological entity. Br Heart J. 1975;37:192–5.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  32. Okura Y, Dec GW, Hare JM. A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis. J Am Col Cardiol. 2003;41(2):322–9.

    Article  Google Scholar 

  33. Heart Failure Society of America, Lindenfeld J, Albert NM, Boehmer JP. HFSA 2010 comprehensive heart failure practice guideline. J Card Fail. 2010;16(6):e1–194. Pivotal guideline recommendations.

    Article  Google Scholar 

  34. Lassner D, Kuhl U, Siegismund CS. Improved diagnosis of idiopathic giant cell myocarditis and cardiac sarcoidosis by myocardial gene expression profiling. Eur Heart J. 2014;35(32):2186–95.

    Article  CAS  PubMed  Google Scholar 

  35. Yancy C, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2013;62(16):e147–239. Pivotal guideline recommendations.

    Article  PubMed  Google Scholar 

  36. Costanzo-Nordin M, Silver M, O’Connell J, et al. Giant cell myocarditis: dramatic hemodynamic histologic improvement with immunosuppressive therapy. Eur Heart J. 1987;76:271–4.

    Article  Google Scholar 

  37. Levy NT, Olson LJ, Weyand C, et al. Histologic and cytokine response to immunosuppression in giant-cell myocarditis. Ann Int Med. 1998;128:648–50.

    Article  CAS  PubMed  Google Scholar 

  38. Steinhaus D, Gelfand E, VanderLaan PA, et al. Recovery of giant-cell myocarditis using combined cytolytic immunosuppression and mechanical circulatory support. J Heart Lung Transplant. 2014;33(7):769–71.

    Article  PubMed  Google Scholar 

  39. Desjardins V, Pelletier G, Leung TK, Waters D. Successful treatment of severe heart failure caused by idiopathic giant cell myocarditis. Can J Cardiol. 1992;8:788–92.

    CAS  PubMed  Google Scholar 

  40. Cooper Jr LT, Hare JM, Tazelaar HD, et al. Giant cell myocarditis treatment trial investigators. Usefulness of immunosuppression for giant cell myocarditis. Am J Cardiol. 2008;102:1535–9. First and only attempt at a randomized controlled trial on treatment of IGCM.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  41. Blauwet LA, Cooper LT. Idiopathic giant cell myocarditis and cardiac sarcoidosis. Heart Fail Rev. 2013;18:733–46.

    Article  PubMed  Google Scholar 

  42. Ankersmit HJ, Ullrich R, Moser B, et al. Recovery from giant cell myocarditis with ECMO support and utilisation of polyclonal antithymocyte globulin: a case report. Thorac Cardiovasc Surg. 2006;54:278–80.

    Article  CAS  PubMed  Google Scholar 

  43. Tayal U, Bell A, Mittal T, et al. Giant cell myocarditis treated with antithymocyte globulin. Br J Hosp Med. 2011;72(8):474.

    Article  Google Scholar 

  44. Marelli D, Kermani R, Bresson J, et al. Support with the BVS 5000 assist device during treatment of acute giant-cell myocarditis. Tex Heart Inst J. 2003;30:50–6.

    PubMed Central  PubMed  Google Scholar 

  45. Murray LK, Gonzalez-Costello J, Jonas SN, et al. Ventricular assist device support as a bridge to heart transplantation in patients with giant cell myocarditis. Eur J Heart Fail. 2012;14(3):312–8.

    Article  PubMed  Google Scholar 

  46. Seeburger J, Doll N, Doll S. Mechanical assist and transplantation for treatment of giant cell myocarditis. Can J Cardiol. 2010;26(2):96–7.

    Article  PubMed Central  PubMed  Google Scholar 

  47. Scott RL, Ratliff NB, Starling RC, et al. Recurrence of giant cell myocarditis in cardiac allograft. J Heart Lung Transplant. 2001;20(3):375–80.

    Article  CAS  PubMed  Google Scholar 

  48. Aguero J, Martinez-Dolz L, Almenar M. Long-term immunosuppressive therapy in recurrent giant cell myocarditis in the transplanted heart: a case report. J Heart Lung Transplant. 2007;39(5):1718–9.

    CAS  Google Scholar 

  49. Toscano G, Tartaro P, Fedrigo M. Rituximab in recurrent giant cell myocarditis after heart transplantation: a potential therapeutic approach. Transpl Int. 2014;27(5):e38–42.

    Article  CAS  PubMed  Google Scholar 

  50. Singh TP, Rabah R, Cooper LT, et al. Total lymphoid irradiation: new therapeutic option for refractory giant cell myocarditis. J Heart Lung Transplant. 2004;23(4):492–5.

    Article  PubMed  Google Scholar 

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Jeffrey A. Shih and Jennifer A. Shih declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Correspondence to Jeffrey A. Shih.

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This article is part of the Topical Collection on Management of Heart Failure

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Shih, J.A., Shih, J.A. Small Steps for Idiopathic Giant Cell Myocarditis. Curr Heart Fail Rep 12, 263–268 (2015). https://doi.org/10.1007/s11897-015-0260-x

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