Congenital anomaly of the tricuspid valve chordae
Synonyms: Congenital anomaly of tricuspid chordae tendineae · Congenital anomaly of tricuspid tendinous chords
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare, congenital anomaly of the tricuspid subvalvular apparatus characterized by aberrant tendinous chords, which insert at the clear zone of the leaflet instead of its free edge and connect to the endocardium instead of the papillary muscles. Resulting tethering of one or more tricuspid leaflets leads to their impaired mobility and tricuspid regurgitation. Association with other congenital cardiac anomalies has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital anomaly of the tricuspid valve chordae | Congenital anomaly of tricuspid chordae tendineae · Congenital anomaly of tricuspid tendinous chords |
| fr | Insertion anormale congénitale des cordages de la tricuspide | |
| de | Kongenitale Anomalie der Chordae tendineae der Trikuspidalklappe | Kongenitale Anomalie der trikuspidalen Sehnenfäden · Kongenitale Anomalie der trikuspidalen Chordae tendineae |
| es | Anomalía congénita de las cuerdas de la válvula tricúspide | Anomalía congénita de las cuerdas tendinosas de la válvula tricúspide |
| it | Anomalia congenita delle corde della valvola tricuspide | |
| nl | Congenitale anomalie van chordae van tricuspidalisklep | Congenitale anomalie van chordae tendineae van tricuspidalisklep · Congenitale anomalie van peesdraden van tricuspidalisklep |
| pt | Anomalia das cordas da válvula tricúspide | |
| pl | Wada strun ścięgnistych zastawki trójdzielnej | = en |
| cs | Anomálie šlašinek trikuspidální chlopně | = en |
| uk | Вроджена аномалія хорд тристулкового клапана | Вроджена аномалія сухожилків трикуспідальних хорд · Вроджена аномалія трикуспідальних сухожильних хорд |
| zh | 先天性三尖瓣腱索异常 | 先天性三尖瓣腱索异常 |
| tr | Triküspit kapak kordasının doğumsal anomalisi | Triküspit tendinöz akorların Doğumsal anomalisi · Triküspit korda tendinelerinin Doğumsal anomalisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q22.8 | NTBT | attributed | validated |
| ICD-11 | LA87.0Y | NTBT | attributed | validated |
| MONDO | 0020396 | E | validated | |
| UMLS | C3165162 | 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:95463Anomaly of the tricuspid subvalvular apparatus
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/99055
- This record as JSON: /data/d/99055.json