ORPHA:423712
Double outlet right ventricle with atrioventricular septal defect, pulmonary stenosis, heterotaxy
Synonyms: DORV with atrioventricular septal defect, pulmonary stenosis, heterotaxy
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Multigenic/multifactorial
- Status
- active
This disease is described under Double outlet right ventricle
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Double outlet right ventricle with atrioventricular septal defect, pulmonary stenosis, heterotaxy | DORV with atrioventricular septal defect, pulmonary stenosis, heterotaxy |
| fr | Ventricule droit à double issue avec communication atrioventriculaire, sténose pulmonaire, hétérotaxie | DORV avec communication atrioventriculaire, sténose pulmonaire, hétérotaxie · VDDI avec communication atrioventriculaire, sténose pulmonaire, hétérotaxie |
| de | Rechter Doppelausstromventrikel mit atrioventrikulärem Septumdefekt, Pulmonalstenose und Heterotaxie | DORV with atrioventrikulärem Septumdefekt, Pulmonalstenose und Heterotaxie |
| es | Doble salida ventricular derecha con defecto septal auriculoventricular, estenosis pulmonar, heterotaxia | DSVD con defecto septal atrioventricular, estenosis pulmonar, heterotaxia |
| it | Ventricolo destro a doppia uscita con difetto del setto atrioventricolare, stenosi polmonare, eterotassia | DORV con difetto del setto atrioventricolare, stenosi polmonare, eterotassia |
| nl | Rechterventrikel met dubbele uitstroom met atrioventriculair septumdefect, pulmonalisklepstenose, heterotaxie | DORV met atrioventriculair septumdefect, pulmonalisklepstenose, heterotaxie |
| pt | Duplicação do trato de saída do ventriculo direito com defeito do septo auriculoventricular, estenose pulmonar, heterotaxia | |
| pl | Dwuodpływowa prawa komora z ubytkiem przegrody przedsionkowo-komorowej, zwężeniem tętnicy płucnej i heterotaksją | DORV z ubytkiem przegrody przedsionkowo-komorowej, zwężeniem tetnicy płucnej i heterotaksją |
| cs | Dvojvýtoková pravá komora s atrioventrikulárním septálním defektem, pulmonální stenózou a heterotaxií | DORV s atrioventrikulárním septálním defektem, pulmonální stenózou a heterotaxií |
| uk | Двостулковий правий шлуночок з дефектом атріовентрикулярної перегородки, легеневий стеноз, гетеротаксис | DORV з дефектом атріовентрикулярної перегородки, стенозом легеневої артерії, гетеротаксисом |
| zh | 异型性右室双出口伴房室间隔缺损及肺动脉狭窄， | 异型性DORV合并房室间隔缺损肺动脉狭窄 |
| tr | Atriyoventriküler septal defekt-pulmoner darlık, heterotaksi ile seyreden çift çıkışlı sağ ventrikül | Atriyoventriküler septal defekt, pulmoner stenozis, heterotaksi ile seyreden DORV |
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.8 | NTBT | attributed | validated |
| MONDO | 0018499 | E | validated | |
| UMLS | C5680071 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:3426Double outlet right ventricle
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare genetic diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
0 recordsNo prevalence record in this release.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/423712
- This record as JSON: /data/d/423712.json