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Giant Cell Myocarditis in Children
1Cardiovascular Division, Mayo Clinic, Rochester, MN.
Insights
Giant cell myocarditis (GCM) is a rare, aggressive heart condition in children, often leading to poor outcomes. Early diagnosis via biopsy is crucial for timely treatment and potential heart transplantation.
Area of Science:
- Pediatric Cardiology
- Immunology
- Cardiovascular Pathology
Background:
- Giant cell myocarditis (GCM) is a rare and aggressive form of myocarditis.
- It presents a significant challenge in pediatric cardiology due to its poor prognosis.
Purpose of the Study:
- To summarize pediatric cases of GCM from a multicenter registry.
- To compare pediatric GCM findings with existing literature.
- To highlight diagnostic and therapeutic considerations for pediatric GCM.
Main Methods:
- Review of 4 pediatric cases (<19 years) from the GCM registry (63 total cases).
- Comparison of clinical presentation, outcomes, and associated conditions with published GCM reports.
- Analysis of diagnostic and therapeutic strategies.
Main Results:
- Pediatric GCM (4/63 cases) typically results in death or heart transplantation, unlike lymphocytic myocarditis.
- Clinical indicators include refractory cardiomyopathy, ventricular arrhythmias, and heart block.
- Associated immune-mediated disorders were observed in females.
Conclusions:
- Prompt endomyocardial biopsy is vital for diagnosing GCM in children.
- Biopsy guides mechanical circulatory support (MCS) and cardiac transplantation decisions.
- Early consideration of immunosuppression (e.g., cyclosporine) is recommended.
Abstract:
Giant cell myocarditis is a rare cause of heart failure and arrhythmias in children. In the multicenter GCM registry, 4 of 63 cases (6%) occurred in subjects less than age 19. In this manuscript, these 4 cases are summarized and the findings related to other published reports. Unlike pediatric lymphocytic myocarditis, that generally has a good prognosis despite a fulminant clinical course, GCM usually results in death or heart transplantation. In children as in adults, GCM can often be distinguished clinically by a failure to respond to usual care and the frequent occurrence of ventricular arrhythmias or heart block in the setting of acute cardiomyopathy. GCM is also associated with other immune-mediated disorders in about 20% of patients. In children associated immune-mediated disorders have only been observed in females. Prompt endomyocardial biopsy in the setting of suspected GCM can affect choice of mechanical circulatory support (MCS), lead to early listing for cardiac transplantation, and consideration of cyclosporine-based immunosuppression.
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