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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 6  |  Issue : 1  |  Page : 84-86

Lymphohistiocytic myocarditis: A rare case with imaging and pathological finding


1 Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India

Date of Submission22-Oct-2019
Date of Decision12-Jan-2020
Date of Acceptance21-Jan-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Priya Jagia
Room No 10A, Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_57_19

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  Abstract 


Lymphohistiocytic myocarditis (LCM) is a rare kind of inflammatory myocardial disease that presents with a wide variety of nonspecific clinical characteristics and requires a high index of suspicion for prompt diagnosis and treatment. The diagnosis of this rare entity is essential to start the immunosuppressive therapy to control the disease process at an early stage. Typical features of layered myocardial enhancement after gadolinium injection in cardiac magnetic resonance (CMR) can suggest the diagnosis of either granulomatous or lymphohistiocytic infiltration of the heart, and biopsy is often required to differentiate between these two rare entities. We present a case of middle-aged women with cardiac conduction block with typical features of LCM in CMR and biopsy.

Keywords: Cardiac magnetic resonance, giant-cell myocarditis, lymphohistiocytic myocarditis, multilayered late gadolinium enhancement


How to cite this article:
Shaw M, Nakra T, Arava SK, Jagia P, Ray R, Chandrashekhara S H, Seth S. Lymphohistiocytic myocarditis: A rare case with imaging and pathological finding. J Pract Cardiovasc Sci 2020;6:84-6

How to cite this URL:
Shaw M, Nakra T, Arava SK, Jagia P, Ray R, Chandrashekhara S H, Seth S. Lymphohistiocytic myocarditis: A rare case with imaging and pathological finding. J Pract Cardiovasc Sci [serial online] 2020 [cited 2020 May 27];6:84-6. Available from: http://www.j-pcs.org/text.asp?2020/6/1/84/282808




  Introduction Top


Inflammatory myocardial diseases are often viral in etiology, but rarer forms are granulomatous or lymphohistiocytic variety. The diagnosis of this entity is often delayed and thus immunosuppressive therapy if not given in a timely manner can lead to cardiac failure. Contrast-enhanced cardiac magnetic resonance (CMR) can suggest the diagnosis of either granulomatous or lymphocytic infiltration of the heart if lamellated/layered appearance is seen in late gadolinium images. Biopsy and immunochemistry markers can reveal lymphocytic infiltration and help make a confident diagnosis and start the timely therapy.


  Case Report Top


A 51-year-old normotensive female visited our cardiology outpatient department with a complaint of vague left-sided chest pain, palpitation, and dyspnea on exertion (New York Heart Association Grade II) for the past 5 months. The symptoms were not associated with fever and the patient was afebrile at the time of presentation. She was evaluated and electrocardiogram showed atrioventricular (AV) conduction block. Chest radiograph revealed mild cardiomegaly with left ventricular configuration. An echocardiography showed moderate left ventricular systolic dysfunction with ejection fraction of 30%–35%. Cardiac enzyme (troponin T and brain natriuretic peptide) levels were essentially normal. Subsequently, the patient was advised for CMR imaging (CMRI) [Figure 1] and [Figure 2] for probable etiological diagnosis and to evaluate the cardiac function. CMRI (1.5 T AERA, Seimens Healthineers, Germany) revealed dilatation of the left ventricle (LV) (measuring 5.8 cm in end diastole at basal region) with thinning of the LV basal septal wall. There was global hypokinesia of the LV wall with severe hypokinesia of basal septum and inferolateral wall of the mid and apical regions of LV. T2-weighted image (T2WI) revealed patchy midmyocardial to transmural hyperintensities in the lateral wall of LV in the mid and apical regions. However, T2 mapping sequence (done to better delineate the myocardial edema) showed increased values (74.2 ± 5.3) (mean ± standard deviation) at not only in the lateral wall but also in the anterior wall of LV with much greater involvement than was evident on T2WI. The perfusion images revealed perfusion deficit in the basal septum (transmural) and lateral wall (midmyocardium) of the mid and apical regions of LV. After giving gadolinium contrast (0.2 mmol/kg), late gadolinium enhancement (LGE) was seen at basal septum (transmural), mid and apical septum (subepicardial), anterior and anterolateral wall (subepicardial to midmyocardial), and inferolateral (subendocardial and subepicardial and layered appearance) in the mid and apical regions of LV. The right ventricular (RV) wall did not reveal any LGE. This pattern of LGE was consistent with myocarditis,[1] and the involvement of the multiple layers of the myocardium[2] suggested a possibility of either giant-cell myocarditis (GCM) or lymphocytic myocarditis. The Dotanoc-PET revealed diffusely increased uptake in whole of the LV wall consistent with ongoing inflammation. Involvement of the subendocardial layer with severe hypokinesia of the lateral walls as compared to the rest of the myocardium raised the possibility of associated coronary artery disease. However, the catheter coronary angiogram was normal and an endomyocardial biopsy was taken from basal RV septum. The biopsy specimen showed multiple foci of lymphohistiocytic infiltrates along with eosinophils and occasional giant cell in the myocardium. Areas of myocyte damage and necrosis were also noted. Masson's trichrome stain revealed patchy interstitial and replacement fibrosis. Immunohistochemical markers for leukocyte common antigen (LCA) highlighted lymphocytes and CD68 highlighted histiocytes in inflammatory infiltrates [Figure 3]. Based on these findings, a final diagnosis of lymphohistiocytic myocarditis (LCM) was made.[3]
Figure 1: Cardiac magnetic resonance imaging: (a and b) T2-weighted image shows areas of hyperintensities (arrow) in the anterolateral wall of the left ventricle suggesting edema. (c) T2 mapping color-coded images showing orange to reddish hue with mean value of 74, consistent with left ventricle myocardial edema. (d) Perfusion images shows deficit (arrow) and thinning of basal interventricular septum.

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Figure 2: 15 min late gadolinium enhancement images: (a-c) Short-axis images in the basal, mid, and apical regions (respectively) show transmural late gadolinium enhancement in septum (black arrow) and subepicardial late gadolinium enhancement in inferolateral (white arrow). In midcavity and apical regions, the late gadolinium enhancement is predominantly at subepicardial layer with layered late gadolinium enhancement appearance in inferolateral segment (double-headed arrow mark – late gadolinium enhancement at subendocardial layer and white arrow mark – late gadolinium enhancement in subepicardial layer) with sparing of mid myocardial layer. (d and e) Late gadolinium enhancement noted in the basal (transmural), lateral, and anterior left ventricle wall (subepicardial).

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Figure 3: Photomicrographs of endomyocardial biopsy: (a) Evidence of myocyte damage and necrosis with moderate lymphohistiocytic and eosinophilic (arrow) infiltrates (H and E). (b) Masson's trichrome stain displays interstitial and replacement fibrosis. (c and d) Immunohistochemical markers highlight lymphocytes (leukocyte common antigen) and histiocytes (CD68).

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After discussion of this case in institutional cardiomyopathy meeting (comprising consultants of cardiology, cardiac radiology, cardiac pathology, and nuclear medicine), a final diagnosis of lymphocytic myocarditis was made and the patient was given the following medication: furosemide 25 mg daily, carvedilol 50 mg daily, enalapril 40 mg daily, prednisolone 50 mg daily for 6 weeks followed by 20 mg for next 5 months, and cyclosporine 150 mg daily for 6 months. The patient's symptoms had improved at 2 weeks, echocardiography showed improvement in the left ventricular ejection fraction, and further follow-up CMRI (to be repeated after 6 month) is awaited to see the response to treatment.


  Discussion Top


The patient presented with short duration symptoms at presentation and it was not clear if the patient was suffering from an acute coronary event or from acute myocarditis. As the patient was a middle-aged woman with nonsignificant risk factors, the possibility of acute coronary syndrome was kept low. Myocarditis was considered as one of the diagnoses, but absence of fever and normal cardiac enzymes level was misleading.[4] Coronary angiogram ruled out coronary artery disease and the typical features of subepicardial LGE in CMRI with hyperintensities in T2WI and T2 mapping sequences favored the diagnosis of myocarditis on imaging. However, the involvement of subendocardial layer along with subepicardial layers on the lateral wall of LV was confusing and a literature search revealed that such multilayered appearance[2],[5] can be found in GCM or lymphocytic myocarditis. The differentiation between these two conditions is essential as an aggressive immunosuppression is the treatment of choice in GCM.[6] The cardiac biopsy sought to arrive at a definite diagnosis would guide the treatment. When the biopsy samples were analyzed, the presence of multiple foci of lymphohistiocytic infiltrates and positive immunohistochemical markers for LCA and CD68 confirmed diagnosis of LCM and the patient was started on cardiac protective medications and short-term steroid therapy. LCM can also present as acute cardiac failure and the diagnosis can be elusive. Timely action with rapid diagnostic tests including a myocardial biopsy and initiation of rapid aggressive treatment can help improve the outcome of patients with this disease. The patient is doing clinically well and repeat ECHO at 6 week revealed improvement in LV systolic function from 25%–30% to 35%–40%. A repeat CMRI will be done at 6 month to look for the changes in LGE and edema pattern including T2 mapping values, which will shed some light on the evolution of this entity after treatment.


  Conclusion Top


This case report highlights the imaging appearance of LCM along with need for early endomyocardial biopsy which would be needed for early recognition of LCM and to differentiate it from GCM. The most common manifestations of LCM are ventricular arrhythmias, AV block, and heart failure. The presence of multilayered LGE in CMRI suggests a diagnosis of either LCM or GCM, while true differentiation between them can be done pathologically. The presence of lymphocytic infiltrates along with positive immunohistochemical markers for LCA and CD68 strongly points toward a diagnosis of LCM.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Ethics clearance

Patient permission taken for publication.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mahrholdt H, Goedecke C, Wagner A, Meinhardt G, Athanasiadis A, Vogelsberg H, et al. Cardiovascular magnetic resonance assessment of human myocarditis: A comparison to histology and molecular pathology. Circulation 2004;109:1250-8.  Back to cited text no. 1
    
2.
Sujino Y, Kimura F, Tanno J, Nakano S, Yamaguchi E, Shimizu M, et al. Cardiac magnetic resonance imaging in giant cell myocarditis: Intriguing associations with clinical and pathological features. Circulation 2014;129:e467-9.  Back to cited text no. 2
    
3.
Dominguez F, Kühl U, Pieske B, Garcia-Pavia P, Tschöpe C. Update on myocarditis and inflammatory cardiomyopathy: Reemergence of endomyocardial biopsy. Rev Esp Cardiol (Engl Ed) 2016;69:178-87.  Back to cited text no. 3
    
4.
Berg J, Kottwitz J, Baltensperger N, Kissel CK, Lovrinovic M, Mehra T, et al. Cardiac magnetic resonance imaging in myocarditis reveals persistent disease activity despite normalization of cardiac enzymes and inflammatory parameters at 3-month follow-up. Circ Heart Fail 2017;10. pii: e004262.  Back to cited text no. 4
    
5.
Gotsman I, Keren A. Fulminant Lymphocytic Myocarditis Versus Giant Cell Myocarditis The Clinical Case of the Month; October, 2008. Available from: https://www.escardio.org/Education/E-Learning/Clinical-cases/Myocardial-and-pericardial-diseases/Fulminant-lymphocytic-myocarditis-versus-giant-cell-myocarditis. [Last accessed on 2019 Mar 22].  Back to cited text no. 5
    
6.
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. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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