Idiopathic giant cell myocarditis

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Idiopathic giant cell myocarditis

ORPHA:329874Disease

Also called IGCM

What it is

A rare cardiac disease characterized by rapidly progressing myocarditis of unknown origin that may lead to progressive heart failure, heart block, ventricular arrhythmias, or sudden cardiac death. It is histologically characterized by myocardial necrosis and fibrosis with multinucleated giant cells and inflammatory cell infiltrate. Some patients have associated autoimmune disorders. It is a high-mortality risk condition and patients often require a heart transplant.

Key facts

Age of onset
Adolescent, Adult, Childhood, Infancy
Inheritance
Not applicable
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

ICD-10 codes

I40.8filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MONDO 0018008UMLS C0264856

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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