Right superior vena cava connecting to left-sided atrium
Synonyms: Right SVC connecting to left-sided atrium · Right superior caval vein connecting to left-sided atrium
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare, congenital vascular malformation of the major vessels characterized by the right SVC passing medially and dorsally to the aortic root and draining into the left atrium. Patients usually present a right-to-left systemic venous blood shunt which may manifest with arterial hypoxemia, cyanosis, exercise dyspnea, clubbing of the fingers, palpitations, murmurs and/or potentially fatal brain abscess. Association with other cardiac anomalies has been reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Right superior vena cava connecting to left-sided atrium | Right SVC connecting to left-sided atrium · Right superior caval vein connecting to left-sided atrium |
| fr | Abouchement de la veine cave supérieure droite dans l'oreillette gauche | |
| de | Fehleinmündung der rechten oberen Hohlvene in den linken Vorhof | Rechte VCS verbunden mit dem linken Vorhof |
| es | Vena cava superior derecha conectada a la aurícula izquierda | VCS derecha que conecta con la aurícula izquierda |
| it | Vena cava superiore destra connessa all'atrio sinistro | |
| nl | Rechter vena cava superior verbonden met linkeratrium | Rechter VCS verbonden met linkeratrium · Rechter bovenste holle ader verbonden met linkeratrium |
| pt | Veia cava superior, direita, ligando ao lado esquerdo da aurícula | |
| pl | Prawa żyła główna górna uchodząca do lewego przedsionka | Prawa SVC uchodząca do lewego przedsionka · Prawa żyła główna górna łącząca się z lewostronnym przedsionkiem |
| cs | Pravá horní dutá žíla ústící do levé síně | Pravá VCS ústící do levé síně · Pravá horní dutá žíla napojující se na levostrannou síň |
| uk | Верхня порожниста вена, яка дренується в ліворозташоване передсердя | Права верхня порожниста вена зєднується з лівим передсердям · Правий SVC зєднується з лівим передсердям |
| zh | 右上腔静脉连接左心房 | 右上腔静脉连通左心房 |
| tr | Sol taraftaki atriyuma bağlanan sağ superior vena kava | Sol taraftaki atriyuma bağlanan sağ SVC · Sol taraftaki atriyuma bağlanan sağ süperior kaval ven |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.8 | NTBT | attributed | validated |
| ICD-11 | LA86.Y | NTBT | attributed | validated |
| MONDO | 0020441 | E | validated | |
| UMLS | C0344658 | 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:95498Congenital anomaly of superior vena cava
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system diseases
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/99110
- This record as JSON: /data/d/99110.json