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LETTER TO THE EDITOR  
Year : 2013  |  Volume : 6  |  Issue : 3  |  Page : 378-382
A rare case of giant cell myocarditis with aortic involvement


1 Department of Pathology, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India
2 Department of Medicine, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India
3 Department of Cardiovascular Thoracic Surgery, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra, India

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Date of Web Publication7-Nov-2013
 

How to cite this article:
Shukla S, Acharya S, Wanjari A, Sancheti A, Mahajan S N. A rare case of giant cell myocarditis with aortic involvement. Ann Trop Med Public Health 2013;6:378-82

How to cite this URL:
Shukla S, Acharya S, Wanjari A, Sancheti A, Mahajan S N. A rare case of giant cell myocarditis with aortic involvement. Ann Trop Med Public Health [serial online] 2013 [cited 2020 Aug 4];6:378-82. Available from: http://www.atmph.org/text.asp?2013/6/3/378/121017
Dear Sir,

A 26-year-old otherwise healthy man presented to us with a 15 days' history of rapidly progressive dyspnea. There was no history of chest pain, cough, expectoration, hemoptysis, paroxysmal nocturnal dysnea, ankle swelling, fever, and rash. He had no significant medical history, did not take any medications, and had no allergies. He was an occasional cigarette smoker and nonalcoholic and had no parental history of hypertension or diabetes mellitus.

General examination: Pulse 112/min, regular, low volume, normal character, and peripheral pulsations were well felt; blood pressure 88/56 mm Hg in the right arm and 86/60 mm Hg in the left arm. Jugular venous pressure was raised with 12 cm of water. CVS examination revealed a normal apex beat on palpation. Auscultation revealed a soft S1 and a soft 2/6 apical ejection systolic murmur without any click or radiation. A presumptive diagnosis of acute myocarditis was done, and he was kept on oxygen therapy and ionotropic support.

On investigation, routine hemogram was found to be normal. Cardiac enzymes were negative, the Brain natriuretic peptide( BNP) level was 843, and d-dimer was normal. A urine drug screen, HIV 1/HIV 2 antibodies, and hepatitis viral panel were negative. Kidney and liver function tests were normal. Rheumatoid factor was normal; ANA titer was negative; the angiotensin-converting enzyme level was normal; dengue, Coxsackie virus A, B, and parvovirus B-19 serology were negative. Patient's electrocardiography showed sinus rhythm, low voltage, and poor R waves in inferior and anterior leads. Chest X ray showed pulmonary edema and cardiomegaly, with nodular pattern in the periphery.

Two-dimensional echocardiography showed mildly dialated left atrium and left ventricle. The apex, distal end, and mid septum were found to be hypokinetic. The septum, lateral wall, and posterior wall showed hypertrophy, which extended to the medial mitral annulus and seemed to involve the posterior mitral leaflet extensively, which resulted in compromised coaptation of leaflets. Color Doppler revealed moderate MR. The anterior mitral leaflet was free and moved normally. The left ventricular systolic function was grossly impaired with ejection fraction of 20-25%. The hypertrophied area showed altered echo pattern [Figure 1]. CT angiogram revealed narrowing and thickening of descending thoracic aorta and narrowing of the origin of left subclavian artery [Figure 2] and [Figure 3].
Figure 1: Two-dimensional echo four-chamber view showing septum, lateral, and posterior wall hypertrophy extending and involving the posterior mitral leaflet with a changed echo pattern

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Figure 2: CT angiogram showing localized narrowing of lower descending aorta at the D9-10 level

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Figure 3: CT coronal view showing eccentric aortic wall thickening up to 8 mm and narrowing is also noted at the origin of left subclavian artery

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Endomyocardial biopsy revealed lymphoeosinophilic infiltrates with multiple giant cells and endomyocardial necrosis suggestive of idiopathic giant cell myocarditis [Figure 4], [Figure 5] and [Figure 6].
Figure 4: Hematoxylin and eosin stained slide scan view showing predominantly fibrocollagenous stroma with the presence of multiple multinucleated giant cells in the midst of nonspecific inflammatory infiltrates and endocardial necrosis

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Figure 5: Hematoxylin and eosin stained slide (×10) shows extensive necrotic areas mixed with chronic inflammatory infiltrates and giant cell reactions

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Figure 6: Hematoxylin and eosin stained slide shows (×40) view of intense eosinophilic inflammatory infiltrate

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Despite of the medical management patient's cardiac status did not improve, and he was referred to higher center for further management.


   Discussion Top


Idiopathic giant cell myocarditis (IGCM) was first described by Saltykow. [1] IGCM is a usually fatal disorder that generally affects young healthy individuals, although a minority of cases occur in association with autoimmune disorders. From 1905 until 1987, all cases were described at autopsy and survival was generally less than 3 months from symptom onset. Several patients whose disease was diagnosed by endomyocardial biopsy survived one or more years in association with immunosuppressive treatment. [2]

In the first half of the 20th century, the term "giant cell myocarditis" was used to describe both granulomatous and diffuse inflammatory endomyocardial infiltrates that contained multinucleated giant cells. [3] Idiopathic granulomatous myocarditis with giant cells was described in several case reports as cardiac sarcoidosis or giant cell myocarditis. [4]

Tesluk, first distinguished the well-organized, granulomatous lesions of cardiac sarcoidosis from a diffuse, nongranulomatous infiltrate, which was designated giant cell myocarditis. [5] Most authorities since have considered giant cell myocarditis as a distinct clinical and pathologic entity rather than a virulent form of isolated cardiac sarcoidosis. [6]

IGCM is a pathologic diagnosis. A diffuse or multifocal inflammatory infiltrate consists of lymphocytes admixed with eosinophils and multinucleated giant cells. Myocyte damage must be evident in association with the inflammatory lesion. Various degrees of fibrosis may be seen. The differential diagnosis of IGCM is discussed in [Table 1]. [7]
Table 1: Differential diagnosis of IGCM

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There are other disorders that can be associated with IGCM [Table 2]. [7]
Table 2: Disorders associated with IGCM

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   Approach to a Case Top


The diagnosis of giant cell myocarditis should be considered for all patients with subacute heart failure of unknown cause. The 63 Multicenter Giant Cell Myocarditis Registry patients describe the clinical and epidemiological pattern of IGCM [Table 3]. [8]
Table 3: Epidemiological pattern of IGCM

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Myocarditis should be suspected in all cases of acute onset and rapidly progressive heart failure in young. Common causes for heart failure and arrhythmia ought to be excluded as per standard clinical practice. After a complete history, physical examination, electrocardiogram, and chest radiograph, an echocardiogram is usually done to exclude valvular and pericardial disease and cardiac masses. There are no specific echocardiographic findings to distinguish giant cell myocarditis from other forms of myocarditis, although the rapid decline in ejection fraction that may occur over several days in patients with giant cell myocarditis is uncommon in lymphocytic myocarditis or cardiac sarcoidosis. Vascular involvement with giant cell myocarditis is a rare association but is a known entity. Pathology remains the same. [9] Other conditions such as Takayasu's arteritis, syphilis, and collagen vascular diseases should be ruled out. Endomyocardial biopsy ought to be considered for patients with heart failure or ventricular arrhythmia of less than 3 months duration who fail to improve despite optimal medical care. In most cases of lymphocytic myocarditis, the left ventricular ejection fraction improves with usual care whereas the ejection fraction in giant cell myocarditis rarely improves. [10] The development of ventricular tachycardia or heart block further increases the likelihood of giant cell myocarditis. The presence of associated disorders such as thymoma, myasthenia gravis, myositis, or inflammatory bowel disease may provide valuable clues.


   Treatment Top


Giant cell myocarditis is rapidly progressive and frequently requires the concurrent management of congestive heart failure, tachyarrhythmias, heart block, and secondary renal and hepatic insufficiency. Supportive care may include standard pharmacologic therapy for congestive heart failure, a permanent or temporary pacemaker, an implantable cardiac defibrillator, and an intra-aortic balloon pump. The use of these drugs and devices should be dictated by standard clinical practice. The treatment of choice is heart transplantation. Ventricular-assist devices have been used to bridge the time until patients with giant cell myocarditis have heart transplantation. Several case reports and the Giant Cell Myocarditis Registry suggest that treatment with certain combinations of immunosuppressants and steroids prolongs transplant-free survival. [11] The usual immunosupressants used are cyclosporine, azathioprine, muromonab CD-3, immunoglobulin, cyclophosphamide, tacrolimus, mycophenolate, or antithymocyte globulin in various combinations.


   Conclusion Top


IGCM is one of the most catastrophic forms of myocarditis that affects heart. High index of suspicion should always be kept in mind while dealing cases of rapidly developing fulminant heart failure in appropriate clinical settings. Other causes of myocarditis should be ruled out. Endomyocardial biopsy is the gold standard investigation and features suggestive of myocardial degeneration and necrosis along with giant cells are the gold standard findings in histopathology. Heart transplantation is the treatment of choice.

 
   References Top

1.Cooper LT Jr. Idiopathic giant cell myocarditis. Myocarditis 2003;405-20.  Back to cited text no. 1
    
2.Menghini VV, Savcenko V, Olson LJ, Tazelaar HD, Dec GW, Kao A, et al. Combined immunosuppression for the treatment of idiopathic giant cell myocarditis. Mayo Clin Proc 1999;74:1221-6.  Back to cited text no. 2
    
3.Kean BH, Hoekenga MT. Giant cell myocarditis. Am J Pathol 1952;28:1095-105.  Back to cited text no. 3
    
4.Scotti TM, McKeown CA. Sarcoidosis involving the heart; report of case with sudden death. Arch Pathol (Chic) 1948;46:289-300.  Back to cited text no. 4
    
5.Tesluk H. Giant cell versus granulomatous myocarditis. Am J Clin Pathol 1956;26:1326-33.  Back to cited text no. 5
    
6.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;9:1126-34.  Back to cited text no. 6
    
7.Cooper LT Jr. Giant cell myocarditis: diagnosis and treatment. Herz 2000;25:291-8.  Back to cited text no. 7
    
8.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. 8
    
9.Roberts WC, Wibin EA. Idiopathic panaortitis, supra-aortic arteritis, granulomatous myocarditis and pericarditis. A case of pulseless disease and possibly left ventricular aneurysm in the African. Am J Med 1966;41:453-61.  Back to cited text no. 9
    
10.Davidoff R, Palacios I, Southern J, Fallon JT, Newell J, Dec GW. Giant cell versus lymphocytic myocarditis. A comparison of their clinical features and long-term outcomes. Circulation 1991;83:953-61.  Back to cited text no. 10
    
11.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.  Back to cited text no. 11
    

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Correspondence Address:
Sourya Acharya
Department of Medicine, JN Medical College, DMIMS University, Sawangi (Meghe), Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1755-6783.121017

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
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