Tricuspid valve agenesis
Synonyms: Congenital unguarded tricuspid orifice
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare, congenital, non-syndromic heart malformation characterized by partial or complete absence of tricuspid valve tissue and its apparatus, with an existing orifice. It can be isolated or associated with other heart anomalies. Clinical presentation is variable and may include syncope, arrhythmias, cyanosis, right heart dilatation and failure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Tricuspid valve agenesis | Congenital unguarded tricuspid orifice |
| fr | Agénésie des valves tricuspides | |
| de | Trikuspidalklappen-Agenesie | Trikusipdalatresie, kongenitale |
| es | Agenesia de la válvula tricúspide | Orificio tricúspide descubierto congénito |
| it | Agenesia della valvola tricuspide | Orifizio tricuspidale pervio congenito |
| nl | Tricuspidalisklepagenesie | Congenitale onbewaakte tricuspidalisklepopening |
| pt | Agenesia da válvula tricúspide | |
| pl | Agnezja zastawki trójdzielnej | Wrodzony brak zastawki trójdzielnej |
| cs | Ageneze trikuspidální chlopně | = en |
| uk | Агенезія тристулкового клапана | Вроджений незахищений тристулковий отвір |
| zh | 三尖瓣发育不全 | 先天性三尖瓣无挡畸形 |
| tr | Triküspit kapak agenezisi | Doğumsal korumasız triküspit orifis |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q22.4 | NTBT | attributed | validated |
| ICD-11 | LA87.0Y | NTBT | attributed | validated |
| MONDO | 0019811 | E | validated | |
| UMLS | C0685715 | 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:98721Congenital tricuspid malformation
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/95457
- This record as JSON: /data/d/95457.json