Idiopathic Giant-Cell Myocarditis — Natural History and Treatment

Cooper, Leslie T.; Berry, Gerald J.; Shabetai, Ralph

doi:10.1056/nejm199706263362603

Cited by 637 publications

(409 citation statements)

References 30 publications

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“…The rapidly accelerating course demanded further cardiac investigation with endomyocardial biopsy with a leading diagnosis of GCM. Previous studies cited steroids, azathioprine, and cyclosporine as immunosuppressive agents for treatment of GCM;5, 6, 8 however, after discussion with experts in GCM, mycophenolate mofetil and tacrolimus were used given the high likelihood of heart transplant in our case.…”

Section: Discussionmentioning

confidence: 82%

“…For this reason, timely referral to centres with a heart failure service and capability of advanced heart failure support is essential. Importantly, recurrence in the transplanted heart is also reported and warrants close follow‐up and routine biopsies 5, 8…”

Section: Discussionmentioning

confidence: 98%

“…Giant cell myocarditis presents with fulminant heart failure (41–75%), refractory ventricular arrhythmias (14–17%), angina‐like symptoms (6–9%), and/or conduction system abnormalities (5–28%) 5, 6. While the exact pathogenesis is unknown, CD‐4 T lymphocyte‐mediated inflammation of the myocardium is suggested 5, 7.…”

Section: Discussionmentioning

confidence: 99%

“…While the exact pathogenesis is unknown, CD‐4 T lymphocyte‐mediated inflammation of the myocardium is suggested 5, 7. There is a 20% incidence of concomitant autoimmune disorder, but only a handful of cases are associated with orbital myositis.…”

Section: Discussionmentioning

confidence: 99%

“…Treatment requires a multimodality approach with pharmacologic interventions and mechanical support devices. Survival without treatment is dismal with a rate of death or cardiac transplantation of 89% and a medial survival of about 5.5 months from symptom onset 5. With combination immunosuppressive agents, survival without need for transplant is drastically improved with a 1 year survival of 77–80% and a 5 year survival of 58% 6, 8.…”

Section: Discussionmentioning

confidence: 99%

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Giant cell myocarditis masquerading as orbital myositis with a rapid, fulminant course necessitating mechanical support and heart transplantation

et al. 2017

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Giant cell myocarditis (GCM), a rapidly progressive inflammation of the myocardium, is associated with fulminant heart failure, refractory ventricular arrhythmias, and conduction system abnormalities. Few case reports have noted orbital myositis as the initial clinical presentation. Our case demonstrates a unique presentation of GCM with only ocular symptoms, which unlike prior studies, rapidly progressed to heart failure, tachyarrhythmias, and conduction disease. Our case necessitated quick recognition and treatment with mechanical support making this the first known case of GCM with successful placement of biventricular assist devices and ultimately with heart transplantation.

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Section: Discussionmentioning

confidence: 82%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Giant cell myocarditis masquerading as orbital myositis with a rapid, fulminant course necessitating mechanical support and heart transplantation

et al. 2017

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Diagnosis and Treatment of Acute Myocarditis

Ammirati

Moslehi

2023

JAMA

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ImportanceAcute myocarditis, defined as a sudden inflammatory injury to the myocardium, affects approximately 4 to 14 people per 100 000 each year globally and is associated with a mortality rate of approximately 1% to 7%.ObservationsThe most common causes of myocarditis are viruses, such as influenza and coronavirus; systemic autoimmune disorders, such as systemic lupus erythematosus; drugs, such as immune checkpoint inhibitors; and vaccines, including smallpox and mRNA COVID-19 vaccines. Approximately 82% to 95% of adult patients with acute myocarditis present with chest pain, while 19% to 49% present with dyspnea, and 5% to 7% with syncope. The diagnosis of myocarditis can be suggested by presenting symptoms, elevated biomarkers such as troponins, electrocardiographic changes of ST segments, and echocardiographic wall motion abnormalities or wall thickening. Cardiac magnetic resonance imaging or endomyocardial biopsy are required for definitive diagnosis. Treatment depends on acuity, severity, clinical presentation, and etiology. Approximately 75% of patients admitted with myocarditis have an uncomplicated course, with a mortality rate of approximately 0%. In contrast, acute myocarditis that is complicated by acute heart failure or ventricular arrhythmias is associated with a 12% rate of either in-hospital mortality or need for heart transplant. Approximately 2% to 9% of patients have hemodynamic instability, characterized by inability to maintain adequate end-organ perfusion, and require inotropic agents, or mechanical circulatory devices, such as extracorporeal life support, to facilitate functional recovery. These patients have an approximately 28% rate of mortality or heart transplant at 60 days. Immunosuppression (eg, corticosteroids) is appropriate for patients who have myocarditis characterized by eosinophilic or giant cell myocardial infiltrations or due to systemic autoimmune disorders. However, the specific immune cells that should be targeted to improve outcomes in patients with myocarditis remain unclear.Conclusions and RelevanceAcute myocarditis affects approximately 4 to 14 per 100 000 people per year. First-line therapy depends on acuity, severity, clinical presentation, and etiology and includes supportive care. While corticosteroids are often used for specific forms of myocarditis (eg, eosinophilic or giant cell infiltrations), this practice is based on anecdotal evidence, and randomized clinical trials of optimal therapeutic interventions for acute myocarditis are needed.

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Choroidal Infiltrate of Unknown Cause

McKay

Sobrin

2019

JAMA Ophthalmol

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A woman in her late 50s reported right eye visual field changes. Review of medical history revealed lumpectomy and radiotherapy for breast cancer 6 years prior. Visual acuity was 20/160 OD (her baseline due to amblyopia) and 20/20 OS. Pupillary function was normal. The anterior chamber and vitreous were quiet. Fundus photographs showed optic nerve edema and a peripapillary choroidal infiltrate in the right eye. Swept-source optical coherence tomography (OCT) demonstrated retinal thickening with cystoid abnormalities, subretinal hyperreflective material consistent with fibrosis, and choroidal thickening in the right eye (Figure 1). Optical coherence tomography of the optic nerve head in the right eye demonstrated 360°peripapillary nerve fiber thickening and diffuse nerve head elevation. Fluorescein angiography demonstrated staining with minimal leakage in the temporal peripapillary retina of the right eye.Testing results for an underlying neoplastic, inflammatory, or infectious process were unrevealing, including negative results of serology tests for tuberculosis, toxoplasmosis, syphilis, Lyme disease, and sarcoidosis. Results of complete blood cell count, serum protein electrophoresis, and bone marrow biopsy were not supportive of lymphoproliferative disease. Results of magnetic resonance imaging of the brain and orbits were normal. Computed tomographic scan of the chest, abdomen, and pelvis demonstrated no lymphadenopathy.Four months prior to presentation, routine electrocardiogram demonstrated a right bundle branch block. Eight months later, complete heart block necessitated urgent pacemaker implantation.

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Idiopathic Giant-Cell Myocarditis — Natural History and Treatment

Cited by 637 publications

References 30 publications

Giant cell myocarditis masquerading as orbital myositis with a rapid, fulminant course necessitating mechanical support and heart transplantation

Giant cell myocarditis masquerading as orbital myositis with a rapid, fulminant course necessitating mechanical support and heart transplantation

Diagnosis and Treatment of Acute Myocarditis

Choroidal Infiltrate of Unknown Cause

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