Idiopathic giant cell myocarditis

General Information (adopted from Orphanet):

Synonyms, Signs: IGCM
Number of Symptoms 31
OrphanetNr: 329874
OMIM Id:
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance:
Age of onset: Adolescent
Adult
25895034 [IBIS]

Disease classification (adopted from Orphanet):

Parent Diseases: Cardiomyopathy
 -Rare cardiac disease

Comment:

Idiopathic giant cell myocarditis is characterized by myocardial inflammation and necrosis leading to rapidly progressive heart failure. In addition to genetic factors autoimmune responses or infections may play a role in the pathogenesis of the disease. Diagnosis should be confirmed by endomyocardial biopsy. (PMID:25895034)

Symptom Information: Sort by abundance 

1
 (HPO:0002014) Diarrhea 24444397 IBIS 225 / 7739
2
 (HPO:0002018) Nausea 24444397 IBIS 44 / 7739
3
 (HPO:0002094) Dyspnea 24444397 IBIS 132 / 7739
4
 (HPO:0005180) Tricuspid regurgitation 12847057 IBIS 20 / 7739
5
 (HPO:0001644) Dilated cardiomyopathy 16844247 IBIS 141 / 7739
6
 (HPO:0001700) Myocardial necrosis 25895034 IBIS 6 / 7739
7
 (HPO:0012664) Reduced ejection fraction 16844247 IBIS 32 / 7739
8
 (HPO:0011712) Right bundle branch block 12847057 IBIS 34 / 7739
9
 (HPO:0001962) Palpitations 9197214 IBIS 62 / 7739
10
 (HPO:0005110) Atrial fibrillation 16844247 IBIS 71 / 7739
11
 (HPO:0004756) Ventricular tachycardia Occasional [IBIS] 9197214 IBIS 55 / 7739
12
 (HPO:0004308) Ventricular arrhythmia 26275338 IBIS 46 / 7739
13
 (HPO:0001663) Ventricular fibrillation Occasional [IBIS] 25895034 IBIS 35 / 7739
14
 (HPO:0001678) Atrioventricular block Frequent [IBIS] 25895034 IBIS 59 / 7739
15
 (HPO:0001635) Congestive heart failure Frequent [IBIS] 75% (n=63) 9197214 IBIS 232 / 7739
16
 (HPO:0002615) Hypotension 24444397 IBIS 52 / 7739
17
 (HPO:0001645) Sudden cardiac death 26275338 IBIS 84 / 7739
18
 (HPO:0001279) Syncope 9197214 IBIS 94 / 7739
19
 (HPO:0012398) Peripheral edema 9197214 IBIS 12 / 7739
20
 (MedDRA:10062049) Lymphocytic infiltration 25895034 IBIS 9 / 7739
21
 (HPO:0009071) Inflammatory myopathy 12535829 IBIS 2 / 7739
22
 (HPO:0003546) Exercise intolerance 9197214 IBIS 62 / 7739
23
 (HPO:0100749) Chest pain 24444397 IBIS 92 / 7739
24
 (HPO:0001945) Fever 24444397 IBIS 218 / 7739
25
 (HPO:0100519) Anuria 24444397 IBIS 20 / 7739
26
 (HPO:0030149) Cardiogenic shock 16844247 IBIS 4 / 7739
27
 (HPO:0012722) Heart block Rare [IBIS] 5% (n=63) 9197214 IBIS 5 / 7739
28
 (HPO:0012819) Myocarditis 26275338 IBIS 4 / 7739
29
 (HPO:0012764) Orthopnea 9197214 IBIS 9 / 7739
30
 (MedDRA:10007548) Cardiac enzymes increased 16844247 IBIS 4 / 7739
31
 (OMIM) Decreased left ventricular ejection fraction (reported in 1 patient) 16844247 IBIS 2 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: