Anomalous aortic origin of coronary artery
Synonyms: AAOCA
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
A rare group of coronary artery congenital malformation disorders characterized by an anomalous origin and course of the left or right coronary artery, which originates from the contralateral aortic sinus of Valsalva and has an anomalous trajectory which may be: pre-pulmonary (with no hemodynamic consequences), retroaortic (with a course posterior to the aortic root and no hemodynamic consequences), interarterial (located between the aorta and the pulmonary artery and associated with a poorer prognosis), subpulmonary (with an intraconal or intraseptal course), or retrocardiac (located in the posterior atrioventricular sulcus). Clinical manifestations depend on the specific anomalous origin and course which is present, with patients being frequently asymptomatic, although nonspecific chest pain, palpitations, dizziness, dyspnea or syncope, usually following physical exertion, may be associated. Sudden death, due to compression/occlusion of the coronary artery and usually associated with, or immediately following, vigorous physical exercise, may be occasionally observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anomalous aortic origin of coronary artery | AAOCA |
| fr | Anomalie d'origine d'une artère coronaire à partir de l'aorte | = en |
| de | Anomaler Abgang der Koronararterie | = en |
| es | Origen anómalo de las arterias coronarias | = en |
| it | Origine anomala dell'arteria coronaria dall'aorta | |
| nl | Aberrante oorsprong van kransslagader in aorta | = en |
| pt | Artéria coronária com origem aórtica anómala | |
| pl | Nieprawidłowe odejście tętnicy wieńcowej od aorty | = en |
| cs | Anomální aortální odstup koronární tepny | = en |
| uk | Аномальне відходження коронарної артерії від аорти | = en |
| zh | 主动脉起源性冠状动脉起源异常 | = en |
| tr | Koroner arterin anormal aort orijini | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| UMLS | C5680159 | 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:1081Coronary artery congenital malformation
rare developmental anomalies during embryogenesis; rare cardiac malformations
Codes that Orphanet moved or referred to this one:
- ORPHA:95493OBSOLETE: Abnormal origin or aberrant course of coronary artery referred to · inactive
- ORPHA:99085OBSOLETE: Coronary artery intramyocardial course referred to · inactive
- ORPHA:99086OBSOLETE: Aortopulmonary coronary arterial course referred to · inactive
- ORPHA:99088OBSOLETE: Intramural coronary arterial course referred to · inactive
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/541478
- This record as JSON: /data/d/541478.json