Double-orifice mitral valve
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Status
- active
A rare, congenital, non-syndromic heart malformation characterized by a single fibrous annulus with two orifices opening into the left ventricle. Clinical presentation is variable and related to the degree of resulting mitral insufficiency and/or stenosis, and depending on the associated heart disease, most commonly atrioventricular septal defect, obstructive left-sided lesions, and cyanotic heart disease. Rare cases of isolated disease have been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Double-orifice mitral valve | |
| fr | Double orifice de la valve mitrale | |
| de | Double-orifice-Mitralklappe | |
| es | Doble orificio valvular mitral | |
| it | Valvola mitrale con doppio orifizio | |
| nl | Dubbel ostium van mitralisklep | Dubbel orificium van mitralisklep |
| pt | Duplo orifício da válvula mitral | |
| pl | Dwuujściowa zastawka mitralna | |
| cs | Dvojité ústí mitrální chlopně | |
| uk | Двуотворний мітральний клапан мітральний клапан | |
| zh | 双孔二尖瓣 | |
| tr | Çift delikli mitral kapak |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q23.8 | NTBT | attributed | validated |
| ICD-11 | LA87.1Y | NTBT | index | validated |
| MONDO | 0019819 | E | validated | |
| UMLS | C0344770 | 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:95465Cleft mitral valve
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/95474
- This record as JSON: /data/d/95474.json