J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website http://www.journalmc.org

Case Report

Volume 4, Number 12, December 2013, pages 775-779

Infiltrative Disease in Non-Ischemic Cardiomyopathy: Giant Cell Myocarditis Leading to Fulminant Myocarditis

Figure 1. Heart, apical core biopsy. Section of myocardial biopsy shows extensive myocardial necrosis and chronic inflammation with lymphocytes, eosinophils, and many giant cells. No granulomas are identified. Giant Cell Myocarditis 4 × H & E Stain

Figure 2. Giant Cell Myocarditis 10 × CD4. Giant Cell Myocarditis 10 × CD8. Heart, apical core biopsy. CD4 and CD8 immunostains show a mixture of CD4 and CD8 positive T cells (CD4 > CD8). Histologic findings are consistent with diagnosis of giant cell myocarditis. Absence of granulomas with extensive necrosis and presence of eosinophils argue against the possibility of sarcoidosis. GMS, AFB stains are negative for fungi and acid fast bacilli.

**Table 1.** Classification of Different Subtypes of Myocarditis
Parameters Considered	Fulminant	Acute	Chronic active	Chronic persistent
DCM, dilated cardiomyopathy; LV, left ventricle; Permission obtained from Elsevier Ltd@ Lieberman EB et al (1991) J Am Coll Cardiol 18: 1617 – 1626.
Onset of cardiac symptoms	Abrupt	Insidious	Insidious	Insidious
Initial presentation	Cardiogenic shock	Heart failure with LV dysfunction	Heart failure with LV dysfunction	Asymptomatic and no LV dysfunction
Initial endomyocardial biopsy findings	Multiple foci of active myocarditis	Active or borderline myocarditis	Active or borderline myocarditis	Active or borderline myocarditis
Clinical course	Complete recovery or death	Incomplete recovery or chronic, stable DCM	Progressive end-stage DM	Mild symptoms with stable LV function
Histologic course	Complete resolution	Complete resolution	Ongoing or resolving myocarditis; fibrosis and giant cells	Persistent inflammation with foci of myocyte necrosis
Response to immunosuppressive therapy	No benefit	Variable	Initial response followed by relapses	No benefit

Table 1. Classification of Different Subtypes of Myocarditis

Parameters Considered

Fulminant

Acute

Chronic active

Chronic persistent

DCM, dilated cardiomyopathy; LV, left ventricle; Permission obtained from Elsevier Ltd@ Lieberman EB et al (1991) J Am Coll Cardiol 18: 1617 – 1626.

Onset of cardiac symptoms

Abrupt

Insidious

Initial presentation

Cardiogenic shock

Heart failure with LV dysfunction

Asymptomatic and no LV dysfunction

Initial endomyocardial biopsy findings

Multiple foci of active myocarditis

Active or borderline myocarditis

Clinical course

Complete recovery or death

Incomplete recovery or chronic, stable DCM

Progressive end-stage DM

Mild symptoms with stable LV function

Histologic course

Complete resolution

Ongoing or resolving myocarditis; fibrosis and giant cells

Persistent inflammation with foci of myocyte necrosis

Response to immunosuppressive therapy

No benefit

Variable

Initial response followed by relapses

No benefit

**Table 2.** Differential for Fulminant Myocarditis
Differential diagnosis	Associated conditions	Key clinical features	Key histologic findings	Treatment
Sachin G, Markham DW, Drazner MH, Mammen PP. Fulminant Myocarditis. Nat Clin Prac. 2008; 5: 693-706.
Giant cell myocarditis	Autoimmune disorders Thymoma Drug hypersensitivity to minocycline, cephalosporins, phenytoin, primidone	Malignant ventricular tachycardia Heart block	Multinucleated giant cells	High-dose steroids and cyclosporine or azathioprine Cardiac transplantation
Necrotizing eosinophilic myocarditis	Allergic diathesis Churg-Strauss syndrome Idiopathic hypereosinophilic syndrome Endomyocardial fibrosis	Flu-like symptoms Peripheral blood eosinophilia	Diffuse infiltration with eosinophils and extensive myocyte necrosis	High-dose steroids
Sarcoidosis	Autoimmune disorders	Arrhythmias Hilar adenopathy	Noncaseating granulomas	High-dose steroids Cardiac transplantation
Peripartum cardiomyopathy	None	Time of onset related to pregnancy	Mild-to-moderate lymphocytic infiltration with myocyte necrosis	Spontaneous recovery High-dose steroids and azathioprine LVAD Cardiac Transplantation Bromocriptine
Acute myocardial infarction	Hypertension Diabetes mellitus Peripheral vascular disease Dyslipidemia	Electrocardiographic evidence of ischemia	Thrombus in coronary arteries with myocyte necrosis	Coronary angioplasty IABP LVAD

Table 2. Differential for Fulminant Myocarditis

Differential diagnosis

Associated conditions

Key clinical features

Key histologic findings

Treatment

Sachin G, Markham DW, Drazner MH, Mammen PP. Fulminant Myocarditis. Nat Clin Prac. 2008; 5: 693-706.

Giant cell myocarditis

Autoimmune disorders Thymoma Drug hypersensitivity to minocycline, cephalosporins, phenytoin, primidone

Malignant ventricular tachycardia Heart block

Multinucleated giant cells

High-dose steroids and cyclosporine or azathioprine Cardiac transplantation

Necrotizing eosinophilic myocarditis

Allergic diathesis Churg-Strauss syndrome Idiopathic hypereosinophilic syndrome Endomyocardial fibrosis

Flu-like symptoms Peripheral blood eosinophilia

Diffuse infiltration with eosinophils and extensive myocyte necrosis

High-dose steroids

Sarcoidosis

Autoimmune disorders

Arrhythmias Hilar adenopathy

Noncaseating granulomas

High-dose steroids Cardiac transplantation

Peripartum cardiomyopathy

None

Time of onset related to pregnancy

Mild-to-moderate lymphocytic infiltration with myocyte necrosis

Spontaneous recovery High-dose steroids and azathioprine LVAD Cardiac Transplantation Bromocriptine

Acute myocardial infarction

Hypertension Diabetes mellitus Peripheral vascular disease Dyslipidemia

Electrocardiographic evidence of ischemia

Thrombus in coronary arteries with myocyte necrosis

Coronary angioplasty IABP LVAD