Congenital pulmonary venous return anomaly
Synonyms: Congenital pulmonary venous connection anomaly
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
A rare developmental defect during embryogenesis where some or all of the pulmonary veins drain into the right atrium or the systemic veins, with or without the presence of pulmonary venous obstruction, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure. The two main subtypes are congenital partial pulmonary venous return anomaly (PAPVC), where one or a few of the pulmonary veins are anomalous, and congenital total pulmonary venous return anomaly (TAPVC), where all of the pulmonary veins are anomalous.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Congenital pulmonary venous return anomaly | Congenital pulmonary venous connection anomaly |
| fr | Anomalie congénitale du retour veineux pulmonaire | |
| de | Lungenvenenfehlmündung, kongenitale | |
| es | Retorno venoso pulmonar anómalo congénito | |
| it | Ritorno venoso polmonare anomalo congenito | Connessione venosa polmonare anomala, tipo congenito |
| nl | Congenitale pulmonale veneuze terugstroomanomalie | Congenitale pulmonale veneuze verbindingsanomalie |
| pt | Retorno venoso pulmonar anómalo congénito | |
| pl | Wrodzony nieprawidłowy spływ żyły płucnej | Wrodzona wada połączenia żyły płucnej |
| cs | Vrozená anomálie návratu plicních žil | Vrozená anomálie spojení plicních žil |
| uk | Вроджена аномалія легеневого венозного повернення | Вроджена аномалія легеневого венозного сполучення |
| zh | 先天性肺静脉回流异常 | 先天性肺静脉连接异常 |
| tr | Doğumsal pulmoner venöz dönüş anomalisi | Doğumsal pulmoner venöz bağlantı anomalisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0017705 | E | validated | |
| MONDO | 17705 | E | validated | |
| UMLS | C0265916 | E | validated | |
| GARD | 4599 | E | validated | |
| MedDRA | 10058079 | 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:98729Congenital pulmonary veins anomaly
rare developmental anomalies during embryogenesis; rare cardiac malformations; rare circulatory system diseases
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/3090
- This record as JSON: /data/d/3090.json