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Year : 2013  |  Volume : 56  |  Issue : 1  |  Page : 67-68
Giant cell myocarditis with epicardial and endocardial involvement: An autopsy case report with review of literature

Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Mumbai Central, Mumbai, India

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Date of Web Publication6-Aug-2013

How to cite this article:
Desai HM, Gayathri AP. Giant cell myocarditis with epicardial and endocardial involvement: An autopsy case report with review of literature. Indian J Pathol Microbiol 2013;56:67-8

How to cite this URL:
Desai HM, Gayathri AP. Giant cell myocarditis with epicardial and endocardial involvement: An autopsy case report with review of literature. Indian J Pathol Microbiol [serial online] 2013 [cited 2022 Jan 23];56:67-8. Available from: https://www.ijpmonline.org/text.asp?2013/56/1/67/116158


Giant cell myocarditis is rare and untreated cases are associated with a high mortality rate (89%). [1] It usually affects young healthy individuals or can be associated with autoimmune disorders. [2] Until 1997, the survival rate for patients with giant cell myocarditis was approximately 3 months. However, the survival rate has improved significantly following early diagnosis by endomyocardial biopsy and heart transplant. We herein report an autopsy-proven case of giant cell myocarditis with epicardial and endocardial involvement.

A 52-year-old male came with complaints of high colored urine, yellow discoloration of sclera since 2 weeks and acute breathlessness since 1 day. Examination revealed icterus, raised jugular venous pressure and bilateral coarse crepts. Investigations showed total leucocyte count of 17,800/cmm with 84% polymorphs. Troponin-T was negative. Chest X-ray showed pulmonary edema and cardiomegaly.

At autopsy, the heart weighed 500 g. The posterior surface of the epicardium showed a 3 cm × 1 cm geographic map-like grayish-white area that was raised above the surface with central congestion. The right and left ventricular myocardium and the interventricular septum also showed similar lesions, few of which were extending into the endocardium and epicardium [Figure 1] and [Figure 2]. Grossly, the lungs showed brown indurations and the liver showed nut-meg appearance.
Figure 1: Greyish-white lesion seen involving the left ventricular myocardium extending to the epicardium

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Figure 2: Endocardial involvement highlighted (marked with arrows)

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On microscopy, the heart showed florid lymphocytic infiltrate along with scattered diffuse numerous multinucleate giant cells [Figure 3] and focal areas of necrosis involving the epicardium, myocardium and endocardium. There were occasional ill-defined epithelioid cell granulomas. Special stains (acid fast bacilli, Gomori methanamine silver and per-iodic Schiff reagent) were negative ruling out the possibility of tuberculous and fungal infection, leading to a diagnosis of giant cell myocarditis. Sections from the lung and liver showed features of chronic passive congestion. The final cause of death was given as congestive cardiac failure following giant cell myocarditis.
Figure 3: Microscopy showing florid lymphocyti cinfiltrate with diff use numerous multi nucleate giant cells (H and E, ×100). Inset: High power view showing characteristi c multi nucleate giant cells (marked with
arrows) (H and E, X400)

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Giant cell myocarditis is a highly fatal disorder with a mortality rate of approximately 89% if untreated. [1] There was a large study by Cooper et al. [1] in 1997 where 63 cases were reported in the Multicenter giant cell myocarditis registry. Following that, till date, there have been less than 190 cases of giant cell myocarditis that have been reported.

The patients are usually young healthy individuals between 15 years and 69 years with equal sex incidence and presenting with dyspnea, peripheral edema, palpitations, arrhythmias or sudden cardiac death. [2] Approximately 20% patients have associated autoimmune disorders, mostly inflammatory bowel disease like ulcerative colitis. [2] The exact etiology and pathogenesis is not known. It has been postulated to be autoimmune in nature, mediated by T-lymphocytes and tumor necrosis factor-α, the latter stimulating the multinucleation of macrophages. [3] While studying the disease progression, Nakayama et al.,[4] found that the initial inflammatory lesion involved the epicardium and then spread transmurally. Giant cell myocarditis has to be distinguished from the other forms of myocarditis in view of the difference in prognosis, the former being associated with a poor prognosis. Right ventricular endomyocardial biopsy has been useful in the diagnosis, with a high sensitivity rate of 82-85%. [5] However, five or more specimens need to be studied to rule out sampling error and other causes of myocarditis. Epicardial and endocardial involvement has been reported but the disease is centered mainly in the myocardium. [2] Our case showed significant epicardial and endocardial involvement, which is exceedingly rare.

Despite a recurrence rate of 25% post-transplant, the survival rate is approximately 71% after transplantation, which is comparable with that of patients of cardiomyopathy with heart transplant. [5] Ventricular-assist devices can be used for treatment to bridge the time gap before a heart transplant. Cardiac transplant, combined with immunosuppressive drug therapy, has been advocated in the management of giant cell myocarditis because death occurs within weeks or months after symptom onset.

   References Top

1.Cooper LT Jr, Berry GJ, Shabetai R. Idiopathic giant-cell myocarditis - Natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators. N Engl J Med 1997;336:1860-6.  Back to cited text no. 1
2.Cooper LT Jr. Idiopathic giant cell myocarditis. In: Cooper LT Jr, editor. Myocarditis: From Bench to Bedside. New Jersey: Humana Press; 2003. p. 405-20.  Back to cited text no. 2
3.Sorimachi K, Akimoto K, Tsuru K, Ieiri T, Niwa A. The involvement of tumor necrosis factor in the multinucleation of macrophages. Cell Biol Int 1995;19:547-9.  Back to cited text no. 3
4.Nakayama Y, Kishimoto C, Shioji K, Sasayama S. Significance of pericardial cytokines in giant cell myocarditis in rats: Pathological comparison to viral myocarditis in mice. Jpn Circ J 2000;64:977-81.  Back to cited text no. 4
5.Shields RC, Tazelaar HD, Berry GJ, Cooper LT Jr. The role of right ventricular endomyocardial biopsy for idiopathic giant cell myocarditis. J Card Fail 2002;8:74-8.  Back to cited text no. 5

Correspondence Address:
Heena M Desai
Department of Pathology, TN Medical College and BYL Nair Charitable Hospital, Mumbai central, Mumbai - 08
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.116158

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  [Figure 1], [Figure 2], [Figure 3]

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