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Giant Cell Myocarditis


Presentation

  • The presentation of GCM may mimic that of lupus myocarditis, including an initial response to immunosuppression.[ncbi.nlm.nih.gov]
  • In this case report, we present atypical presentation of atrial giant cell myocarditis with mass lesion, which completely resolved after successful surgical resection without immuno suppression therapy.[ncbi.nlm.nih.gov]
  • Giant cell myocarditis (GCM) is a fatal form of myocarditis that often presents with acute heart failure. An inflammatory infiltrate with giant cells and myocardial necrosis is characteristic.[ncbi.nlm.nih.gov]
  • A review of the natural history and treatment of GCM is also presented.[ncbi.nlm.nih.gov]
  • Herein we present a case of fulminant myocarditis in a woman previously treated for B-cell lymphoma.[ncbi.nlm.nih.gov]
Fatigue
  • Approximately 2 months later, she developed abdominal pain, vomiting, weight gain, and fatigue. She was diagnosed with congestive heart failure and cardiomyopathy, and endomyocardial biopsy revealed giant cell myocarditis.[ncbi.nlm.nih.gov]
  • Symptoms include swelling of the ankles, chest pain, heart palpitations, fatigue, and shortness of breath (lying flat or exerting energy). Patients with the disease develop irregular heartbeats.[knightcrier.org]
  • Case V.P. is a 13-year-old female with no past medical history who presented to an outside hospital with a 3-week history of fatigue, emesis, 10-pound weight loss, headache, cough, and intermittent shortness of breath.[chop.edu]
  • The symptoms vary from simple fatigue to sudden death. Once a diagnosis is made it is easy to look back and connect these symptoms with giant cell myocarditis.[myocarditisfoundation.org]
  • Symptoms can vary and can include fatigue, dyspnea, edema, palpitations, and sudden death. Diagnosis is based on symptoms and clinical findings of abnormal ECG, cardiac biomarkers, and cardiac imaging in the absence of cardiovascular risk factors.[msdmanuals.com]
Death in Infancy
  • This entity can be the cause of some cases of sudden unexpected death in infancy.[ncbi.nlm.nih.gov]
Heart Disease
  • Giant cell myocarditis (GCM) is an uncommon inflammatory heart disease with a rapid progression and a devastating outcome. Its exact cause is unknown, but it has been associated with various inflammatory and autoimmune disorders.[ncbi.nlm.nih.gov]
  • Fulminant Giant Cell Myocarditis (GCM) is a rare autoimmune heart disease that automatically qualified for disability benefits through either of both of the Social Security Administration's (SSA's) disability programs, though you must still complete the[disabilitybenefitscenter.org]
  • ABSTRACT Giant cell myocarditis(GCM) is a rare inflammatory heart disease which is characterized by multinucleated giant cells and a granulomatous reaction. It usually progresses rapidly and results in a fatal course.[jpatholtm.org]
  • Microscopic features: After common causes of heart disease are excluded, the diagnosis of Giant cell myocarditis must be confirmed by endomyocardial biopsy.[histopathology-india.net]
  • Catheterization with coronary angiography was performed and did not reveal significant heart disease, and ventriculography showed a 22% ejection fraction with severe anterolateral and diaphragmatic hypokinesia and slight anterobasal and apical hypokinesia[revespcardiol.org]
Left Ventricular Dysfunction
  • Giant cell myocarditis (GCM) is an uncommon disorder that affects ventricular myocardium causing severe left ventricular dysfunction and ventricular arrhythmias. We report a case of GCM that only affected the atrium sparing the ventricle.[ncbi.nlm.nih.gov]
  • When severe left ventricular dysfunction due to an unknown cause is complicated by HIT, potential diagnoses of giant-cell and other types of autoimmune myocarditis should thus be investigated.[ncbi.nlm.nih.gov]
  • Cardiac catheterization confirmed severe 2-vessel disease and left ventricular dysfunction.[ncbi.nlm.nih.gov]
  • The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.[ncbi.nlm.nih.gov]
Cardiomegaly
  • Most patients described have cardiomegaly with biventricular dilatation, but in this elderly patient with severe coronary artery disease, there was an element of superimposed hypertrophy.[path.upmc.edu]
  • 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.[atmph.org]
  • His chest radiograph and chest computed tomography showed pulmonary congestion and bilateral pleural effusion without cardiomegaly, and the electrocardiogram showed low voltage of R-wave and intraventricular conduction disturbance in all leads (Fig. 1[jaclinicalreports.springeropen.com]
Cardiac Signs and Symptoms
  • The diagnosis of CS may be easy if cardiac signs or symptoms develop in a patient with a known diagnosis of extra-cardiac disease, but is particularly challenging in isolated CS.[escardio.org]
Gallop Rhythm
  • Physical examination revealed cyanosis, difficulty in breathing, gallop rhythm, and shock. She died in refractory congestive heart failure 16 hours after entering the hospital. A thyroidectomy was performed at age 15 years because[jamanetwork.com]
Suggestibility
  • Serological and immunoblotting techniques revealed a myocardial infection with coxsackie B2 virus, suggesting a viral etiology of this disease.[ncbi.nlm.nih.gov]
  • Recent studies suggest that the ventricular arrhythmias in GCM may be mediated by a cytokine-induced change in desmosomal protein expression.[ncbi.nlm.nih.gov]
  • The lymphocytes were predominantly CD4 positive T cells, and the majority of these cells stained with antibodies for perforin, suggesting an unusual cytolytic role for these lymphocytes.[ncbi.nlm.nih.gov]
  • A novel possible trigger was suggested by a positive serologic test for leptospirosis in one case.[ncbi.nlm.nih.gov]
  • The clinico-pathological correlation suggests a link between drug hypersensitivity and the giant cell myocarditis.[ncbi.nlm.nih.gov]
Intracranial Hemorrhage
  • Therefore, we performed a computed tomography (CT) scan of the brain, which revealed the intracranial hemorrhage. He did not recover from the heart failure and died 16 days after the admission.[jaclinicalreports.springeropen.com]

Workup

  • Her other workup of hypercalcemia was unrevealing for vitamin D intoxication and multiple myeloma. Computed tomography of her chest showed no signs of hilar lymphadenopathy.[ncbi.nlm.nih.gov]
  • (See Etiology, Clinical, and Workup.)[emedicine.medscape.com]
Mural Thrombus
  • Among the 5 living patients, echocardiography revealed severe atrial dilatation (5 cases), mitral/tricuspid regurgitation (5), atrial mural thrombus (3), atrial wall thickening (2), and atrial hypokinesis (2).[ncbi.nlm.nih.gov]
Atrioventricular Dissociation
  • The serum creatine phosphokinase (CPK) level was abnormally elevated, and abnormal electrocardiographic findings were noted, including atrioventricular dissociation and ventricular escape contraction.[ncbi.nlm.nih.gov]

Treatment

  • Therefore, despite individual reports of dramatic improvement after immunosuppressive treatment, firm conclusions cannot be made about the benefit of immunosuppression for GCM.[ncbi.nlm.nih.gov]
  • Interleukin-2 (IL-2) is used in the treatment of solid tumors and hematologic malignancies. Sudden death is a rare complication of IL-2 treatment.[ncbi.nlm.nih.gov]
  • Treatment with methylprednisolone and cyclosporine was promptly initiated. Several apical clots were noted during treatment, but the patient attained full recovery with treatment.[ncbi.nlm.nih.gov]
  • Treatment had then been continued with steroids; however, due to subsequent deterioration of his diabetic state, treatment was switched to the immunosuppressant drug tacrolimus.[ncbi.nlm.nih.gov]
  • The only multicenter case series with treatment data lacked cardiac function assessments and had a retrospective design.[ncbi.nlm.nih.gov]

Prognosis

  • Specific histopathologic forms include fulminant lymphocytic myocarditis, which has an excellent prognosis with standard heart failure care.[ncbi.nlm.nih.gov]
  • Despite initial clinical similarities, lupus myocarditis and GCM are histologically distinct entities with dramatic differences in prognosis.[ncbi.nlm.nih.gov]
  • INTRODUCTION: Although prognosis in acute myocarditis is generally moderate, giant cell myocarditis shows poor prognosis.[ncbi.nlm.nih.gov]
  • The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.[ncbi.nlm.nih.gov]
  • The clinical recognition that GCM may be a manifestation of an adverse drug reaction is important, since the prognosis and treatment of this entity may be different from that of other forms of myocarditis.[ncbi.nlm.nih.gov]

Etiology

  • Serological and immunoblotting techniques revealed a myocardial infection with coxsackie B2 virus, suggesting a viral etiology of this disease.[ncbi.nlm.nih.gov]
  • No infectious etiologies, hypersensivity, or autoimmune disorders were identified.[ncbi.nlm.nih.gov]
  • Giant cell myocarditis, the etiology of which is not defined, is a rare disease with unfavorable prognosis. This case suggests the possibility of drug-induced GCM.[ncbi.nlm.nih.gov]
  • The etiology of IGCM is unknown. Endomyocardial biopsy and histological examination are essential for diagnosis. In our case, a one-month-old baby boy with no prior medical history died during breast-feeding.[ncbi.nlm.nih.gov]
  • Histologic examination remains the gold standard for diagnosis; however, there are many possible etiologies for cardiac giant cells.[ncbi.nlm.nih.gov]

Epidemiology

  • No data are available about the epidemiology of GCM in children.[ncbi.nlm.nih.gov]
  • Further systemic investigation of epidemiology, causes, natural history and effects of treatment, including cardiac transplantation is needed. References 1. Cooper LT , Berry GJ , Shabetai R .[nature.com]
  • The 63 Multicenter Giant Cell Myocarditis Registry patients describe the clinical and epidemiological pattern of IGCM [Table 3] . [8] Myocarditis should be suspected in all cases of acute onset and rapidly progressive heart failure in young.[atmph.org]
  • J Am Acad Dermatol 26: 441–448 PubMed Google Scholar Nordborg E (2000) Epidemiology of biopsy-positive giant cell arteritis: an overview.[springerlink.com]
  • […] eosinophilic and giant cell myocarditis (GCM) Terminology GCM has been termed idiopathic GCM, pernicious myocarditis, Fiedler myocarditis, acute idiopathic interstitial myocarditis, giant cell granulomatous myocarditis, and granulomatous myocarditis Epidemiology[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • The underlying pathophysiologic mechanisms are not yet defined but appear to involve genetics, autoimmune disorders, and possibly environmental factors such as viruses.[ncbi.nlm.nih.gov]
  • The coincidence of CVID and GCM in a single patient may reflect a pathophysiologic connection.[ncbi.nlm.nih.gov]
  • Giant cell myocarditis is considered to be an autoimmune disease, however, its pathophysiology and specific treatment is yet to be elucidated.[ncbi.nlm.nih.gov]
  • […] myocarditis, Fiedler myocarditis, acute idiopathic interstitial myocarditis, giant cell granulomatous myocarditis, and granulomatous myocarditis Epidemiology Incidence difficult to determine as most cases are believed to be subclinical or underdiagnosed Pathophysiology[pathologyoutlines.com]

Prevention

  • We conclude that, in the case of nonischemic acute heart failure in young patients, a biopsy should be performed as soon as possible to prevent an unfavourable outcome of this often fatal disease.[ncbi.nlm.nih.gov]
  • While immunosuppressive therapy is usually able to keep under control the disease and prolong the average transplant-free survival in many patients, effective therapeutic strategies to prevent or treat the recurrence of GCM in transplanted organs are[ncbi.nlm.nih.gov]
  • A better understanding of giant cell myocarditis pathogenesis is critical to developing treatments to prevent progression and reverse damage. We compared the gene expression of giant cell myocarditis with that of nonfailing hearts.[ncbi.nlm.nih.gov]
  • To prevent bias, randomization will be stratified by recency of symptom onset to ensure that both the treatment and control groups are balanced with respect to it.[clinicaltrials.gov]
  • Therefore, the cornerstone in prevention of intraventricular thrombosis is systemic anticoagulant therapy and prevention of left ventricular distension.[jaclinicalreports.springeropen.com]

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