Congenital partial agenesis of pericardium
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
Congenital partial agenesis of pericardium is a rare, mostly asymptomatic, congenital heart malformation mainly characterized by the partial absence of the left pericardium. It is occasionally associated with chest pain or dyspnea and is usually incidentally diagnosed during surgery or at autopsy. Herniation and strangulation of a portion of the heart through the pericardial foramen may occur, resulting in myocardial acute ischemia and possible sudden death. Right side pericardium involvement is rare.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital partial agenesis of pericardium | |
| fr | Anomalie congénitale partielle du péricarde | |
| de | Perikardagenesie, kongenitale, partielle | |
| es | Agenesia parcial de pericardio congénita | |
| it | Agenesia parziale congenita del pericardio | |
| nl | Congenitale partiële agenesie van pericard | |
| pt | Agenesia parcial do pericárdio congénita | |
| pl | Wrodzona częściowa agenezja osierdzia | |
| cs | Vrozená částečná ageneze perikardu | |
| uk | Вроджена часткова агенезія перикарда | |
| zh | 先天性心包部分发育不全 | |
| tr | Perikardin doğumsal kısmi agenezi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.8 | NTBT | attributed | validated |
| ICD-11 | LA8D | NTBT | index | validated |
| MONDO | 0020455 | E | validated | |
| UMLS | C0345139 | 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:2846Congenital pericardium anomaly
rare developmental anomalies during embryogenesis; rare cardiac malformations
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/99130
- This record as JSON: /data/d/99130.json