Anomaly of the tricuspid subvalvular apparatus
- Type
- Category
- Group
- Group of disorders
- Status
- active
A group of rare congenital tricuspid malformations characterized by anomalies of the chordae tendineae and papillary muscles, including aberrant chordae tendineae, straddling valve (abnormal attachment of the chordae tendineae to both ventricles), and parachute valve (unifocal attachment of the chordae tendineae to a single or fused papillary muscle), resulting in an incompetent valve with regurgitation and/or stenosis and impaired right ventricular inflow, potentially leading to heart failure. In most cases, other cardiac anomalies are found in association.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anomaly of the tricuspid subvalvular apparatus | |
| fr | Anomalie de l'appareil sous-valvulaire tricuspide | |
| de | Anomalie des subvalvulären Apparates der Trikuspidalklappe | |
| es | Anomalía del aparato subvalvular tricúspide | |
| it | Anomalia dell'apparato subvalvolare tricuspidale | |
| nl | Anomalie van tricuspidalis-subvalvulair apparaat | |
| pt | Anomalia do aparelho tricúspide subvalvular | |
| pl | Anomalie aparatu podzastawkowego zastawki trójdzielnej | |
| cs | Anomálie trikuspidálního subvalvulárního aparátu | |
| uk | Аномалії підклапанного апарату тристулкового клапану | |
| zh | 三尖瓣下结构异常 | |
| tr | Triküspit subvalvüler aparatın anomalisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q22.8 | E | specific | validated |
| UMLS | C5681574 | 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
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/95463
- This record as JSON: /data/d/95463.json