Persistent left superior vena cava connecting to the roof of left-sided atrium
Synonyms: Persistent left SVC connecting to left-sided atrium · Persistent left SVC connecting to the roof of left-sided atrium · Persistent left superior vena cava connecting to left-sided atrium
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare congenital anomaly of superior vena cava characterized by a persistent left superior vena cava that drains into the left atrium through a direct connection to its roof, creating a right-to-left shunt. Patients are at risk of developing chronic hypoxia, decreased exercise tolerance, cyanosis, embolic cerebrovascular events, and heart failure.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Persistent left superior vena cava connecting to the roof of left-sided atrium | Persistent left SVC connecting to left-sided atrium · Persistent left SVC connecting to the roof of left-sided atrium · Persistent left superior vena cava connecting to left-sided atrium |
| fr | Abouchement de la veine cave supérieure gauche dans le toit de l'oreillette gauche | |
| de | Persistenz der linken Vena cava superior mit Verbindung zum dorsalen Bereich des linken Vorhofes | Persistierende LVCS mit Mündung in den linken Vorhof · Persistierende linke obere Hohlvene mit Mündung in den linken Vorhof · Persistierende linke obere Hohlvene mit Verbindung zum dorsalen Bereich des linken Vorhofes |
| es | Vena cava superior izquierda persistente conectada al techo de la aurícula izquierda | VCS izquierda persistente conectada a la aurícula izquierda |
| it | Persistenza della vena cava superiore sinistra connessa al tetto dell'atrio sinistro | |
| nl | Persisterende linker vena cava superior verbonden aan dak van linkeratrium | Persisterende linker VCS verbonden aan dak van linkeratrium · Persisterende linker VCS verbonden aan linkeratrium · Persisterende linker bovenste holle ader verbonden aan linkeratrium · Persisterende linker vena cava superior verbonden aan linkeratrium |
| pt | Veia cava superior esquerda persistente do lado esquerdo da aurícula | |
| pl | Lewa żyła główna górna uchodząca do lewego przedsionka | Lewa SVC uchodząca do lewego przedsionka · Lewa żyła główna wchodząca do lewostronnego przedsionka |
| cs | Levá horní dutá žíla pokračující do levostranné síně | Levá SVC pokračující do levostranné síně · Levá vena cava superior pokračující do levostranné síně |
| uk | Додаткова верхня ліва порожниста вена, що дренується в склепіння ліворозташованого передсердя | Персистуюча ліва верхня порожниста вена зєднується з лівостороннім передсердям · Персистуючий лівий шлуночок, що зєднується з лівим передсердям · Персистуючий лівий SVC, що зєднується з дахом лівого передсердя |
| zh | 永存左上腔静脉连接冠状窦顶 | 永存左SVC连接左心房 |
| tr | Sol taraftaki atriyumun üst tarafına bağlanan kalıcı sol superior vena kava | Sol taraftaki atriyumun çatısına bağlanan kalıcı sol SVC · Sol taraftaki atriyuma bağlanan kalıcı sol süperior vena kava · Sol taraftaki atriyuma bağlanan kalıcı sol SVC |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q26.1 | NTBT | attributed | validated |
| ICD-11 | LA86.0 | NTBT | index | validated |
| MONDO | 0020442 | E | validated | |
| MONDO | 20442 | E | validated | |
| UMLS | C5680292 | 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/99111
- This record as JSON: /data/d/99111.json