Coronary ostial stenosis or atresia
Synonyms: COSA · Congenital coronary arterial orifice stenosis or atresia · Congenital stenosis or atresia of a coronary ostium
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
A rare coronary artery congenital malformation characterized by congenital, partial or total occlusion of the left or right coronary artery orifice, associated with hypoplasia of the proximal segment of the corresponding coronary artery. It may present with failure to thrive, dyspnea, syncope, angina pectoris, ventricular tachycardia, myocardial ischemia and/or sudden death.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Coronary ostial stenosis or atresia | COSA · Congenital coronary arterial orifice stenosis or atresia · Congenital stenosis or atresia of a coronary ostium |
| fr | Sténose ou atrésie ostiale coronaire | Sténose ou atrésie congénitale d'un orifice artériel coronaire · Sténose ou atrésie congénitale d'un ostium coronaire |
| de | Koronare Ostiumstenose oder Atresie | COSA · Koronarostiumstenose oder -atresie, kongenitale |
| es | Estenosis o atresia ostial coronaria | Estenosis o atresia congénita ostial coronaria |
| it | Stenosi o atrofia dell'ostium coronarico | |
| nl | Coronaire ostiale stenose of atresie | COSA · Congenitale stenose of atresie van een coronair ostium · Congenitale stenose of atresie van een coronaire arteriële opening |
| pt | Óstio coronário, estenose ou atrofia | |
| pl | Zwężenie lub zanik ujścia wieńcowego | = en |
| cs | Stenóza nebo atrofie koronárního ostia | = en |
| uk | Стеноз або атрезія вустя коронарної артерії | Вроджений стеноз або атрезія устя коронарної артерії · Вроджений стеноз або атрезія коронарного отвору · COSA |
| zh | 冠状动脉开口狭窄或闭锁 | 先天性冠状动脉口狭窄或闭锁 |
| tr | Koroner ostial stenoz veya atrezi | COSA · Koroner ostiumun doğumsal stenozu veya atrezisi · Doğumsal koroner arter açıklığı darlığı veya atrezi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.5 | NTBT | attributed | validated |
| ICD-11 | LA86.Y | NTBT | index | validated |
| MONDO | 0020423 | E | validated | |
| UMLS | C5575847 | 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:542822Anomaly of the coronary ostia
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/99087
- This record as JSON: /data/d/99087.json