Persistent fifth aortic arch
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare, congenital anomaly of the great arteries characterized by an extrapericardial vessel arising from the ascending aorta proximal to the brachiocephalic artery and terminating either in the dorsal aorta or in pulmonary arteries via a persistently patent arterial duct. The resulting connection is a systemic-to-systemic or systemic-to-pulmonary. Clinical manifestation include exercise intolerance, reduced femoral pulses, cyanosis with or without pulmonary hypertension and heart failure. Other congenital cardiovascular anomalies are often present and influence the clinical presentation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Persistent fifth aortic arch | |
| fr | Persistance du cinquième arc aortique | |
| de | Aortenbogen, fünfter persistierender | |
| es | Persistencia del quinto arco aórtico | |
| it | Persistenza del quinto arco aortico | |
| nl | Persistente vijfde aortaboog | |
| pt | Quinto arco aórtico persistente | |
| pl | Przetrwały piąty łuk aorty | |
| cs | Perzistující pátý aortální oblouk | |
| uk | Персистуюча пята дуга аорти | |
| zh | 永存第5对主动脉弓 | |
| tr | Kalıcı beşinci aort arkı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q25.4 | NTBT | attributed | validated |
| ICD-11 | LA8B.2Y | NTBT | index | validated |
| MONDO | 0020414 | E | validated | |
| UMLS | C0345066 | 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/99076
- This record as JSON: /data/d/99076.json