Anomalous aortic origin of the right coronary artery
Synonyms: AORCA · R-ACAOS · Right coronary artery from left aortic sinus
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare coronary artery congenital malformation characterized by an anomalous origin and course of the right coronary artery, which originates from the left aortic sinus of Valsalva and has an abnormal proximal course, which may be intramural, prepulmonic, subpulmonic, retroaortic, retrocardiac or wrapped around the apex. Patients are frequently asymptomatic, although chest pain, dyspnea, palpitations, dizziness, syncope, and sudden cardiac arrest/death (typically following intense physical exertion) may be observed. This malformation is associated with a lower risk of sudden cardiac death therefore surgical revascularization is recommended only when signs and/or symptoms of ischemia are present.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anomalous aortic origin of the right coronary artery | AORCA · R-ACAOS · Right coronary artery from left aortic sinus |
| fr | Anomalie de naissance de l'artère coronaire droite à partir de l'aorte | Anomalie d'origine de l'artère coronaire droite à partir de l'aorte · Naissance de l'artère coronaire droite à partir du sinus gauche · R-ACAOS |
| de | Anomaler Abgang der rechten Koronararterie | AORCA · Fehlabgang der rechten Koronararterie aus dem linken Aortensinus · R-ACAOS |
| es | Origen anómalo de la arteria coronaria derecha | AORCA · Arteria coronaria derecha en el seno aórtico izquierdo · R-ACAOS |
| it | Origine anomala dell'arteria coronaria destra dall'aorta | |
| nl | Aberrante oorsprong van rechterkransslagader in aorta | AORCA · R-ACAOS · Rechterkransslagader uit linker sinus van Valsalva |
| pt | Artéria coronária direita com origem aórtica anómala | |
| pl | Nieprawidłowe odejście prawej tętnicy wieńcowej od aorty | = en |
| cs | Anomální aortální odstup pravé koronární tepny | AORCA · R-ACAOS · Pravá koronární tepna z levého aortálního sinu |
| uk | Аномальне відходження правої коронарної артерії від аорти | AORCA · R-ACAOS · Права коронарна артерія від лівого аортального синуса |
| zh | 主动脉起源性右冠状动脉起源异常 | 右冠状动脉起源于左主动脉窦 |
| tr | Sağ koroner arterin anormal aort orijini | AORCA · R-ACAOS · Sol aort sinüsünden sağ koroner arter |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.5 | NTBT | attributed | validated |
| ICD-11 | LA8C.1 | NTBT | index | validated |
| MONDO | 0035250 | E | validated | |
| UMLS | C3532025 | 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:541478Anomalous aortic origin of coronary artery
rare developmental anomalies during embryogenesis; rare cardiac malformations
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/541454
- This record as JSON: /data/d/541454.json