Encircling double aortic arch
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
Encircling double aortic arch is a very rare congenital anomaly of the great arteries characterized by the presence of two aortic arches (right and left) which encircle and compress the trachea and esophagus, resulting in various respiratory and gastrointestinal symptoms (e.g. harsh breathing, stridor, dyspnea, cyanotic and choking episodes, chronic cough, recurrent respiratory tract infections, dysphagia and reflux). Esophageal atresia and tracheoesophageal fistula have also been reported. It usually occurs isolated, but, on occasion, may be associated with other congenital heart anomalies and chromosomal aberations.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Encircling double aortic arch | |
| fr | Aorte encerclante | |
| de | Doppelter Aortenbogen mit zirkulärer Einengung von Trachea/Ösophagus | |
| es | Anillo vascular por doble arco aórtico | |
| it | Doppio arco aortico | |
| nl | Omcirkelende dubbele aortaboog | |
| pt | Arco aórtico duplo | |
| pl | Podwójny łuk aorty | |
| cs | Dvojitý aortální oblouk | |
| uk | Огинаюча подвійна дуга аорти | |
| zh | 围绕型双主动脉弓 | |
| tr | Çevreleyen çift aortik ark |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q25.4 | NTBT | inclusion | validated |
| ICD-11 | LA8B.Y | NTBT | index | validated |
| MONDO | 0020413 | E | validated | |
| UMLS | C4706940 | 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:1132Aortic arch defects
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/99075
- This record as JSON: /data/d/99075.json