Idiopathic giant cell myocarditis
Synonyms: IGCM
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Infancy
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic giant cell myocarditis | IGCM |
| fr | Myocardite idiopathique à cellules géantes | IGCM · Myocardite idiopathique gigantocellulaire |
| de | Riesenzell-Myokarditis, idiopathische | = en |
| es | Miocarditis idiopática de células gigantes | = en |
| it | Miocardite idiopatica a cellule giganti | = en |
| nl | Idiopathische reuscelmyocarditis | = en |
| pt | Miocardite idiopática de células gigantes | = en |
| pl | Idiopatyczne olbrzymiokomórkowe zapalenie mięśnia sercowego | = en |
| cs | Obrovskobuněčná myokarditida | = en |
| uk | Ідіопатичний гігантоклітинний міокардит | = en |
| zh | 特发性巨细胞心肌炎 | = en |
| tr | İdiyopatik dev hücreli miyokardit | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I40.8 | NTBT | attributed | validated |
| ICD-11 | BC42.0 | E | specific | validated |
| MONDO | 0018008 | E | validated | |
| UMLS | C0264856 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:167848Rare cardiomyopathy
rare cardiac diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
17 annotations · validated 2026-03-27| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0500016 | Abnormal cardiac MRI | |
| HP:0410174 | Increased circulating troponin T concentration | |
| HP:4000004 | Myocardial late gadolinium enhancement | |
| HP:0031324 | Myocardial multinucleated giant cells | |
| HP:0012664 | Reduced left ventricular ejection fraction | |
| Frequent (79-30%) | ||
| HP:0001635 | Congestive heart failure | |
| HP:0031185 | Increased circulating NT-proBNP concentration | |
| HP:0001685 | Myocardial fibrosis | |
| HP:0001700 | Myocardial necrosis | |
| Occasional (29-5%) | ||
| HP:0001678 | Atrioventricular block | |
| HP:0002960 | Autoimmunity | |
| HP:0030149 | Cardiogenic shock | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0003565 | Elevated erythrocyte sedimentation rate | |
| HP:4000141 | Left ventricular dilatation | |
| HP:0001645 | Sudden cardiac death | |
| HP:0004756 | Ventricular tachycardia | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 37734812[PMID]_33660520[PMID]_41558852[PMID]_33632487[PMID]_38431769[PMID]_28745125[PMID]_17916581[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/329874
- This record as JSON: /data/d/329874.json