Anomalous origin of coronary artery from the pulmonary artery
Synonyms: ACAPA
- Type
- Morphological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare coronary artery congenital malformation characterized by an anomalous origin of the left (ALCAPA) or right (ARCAPA) coronary artery from the pulmonary artery, with variable clinical presentation, ranging from asymptomatic to early heart failure and death depending on the degree of development of collateral circulation between the left and right coronary artery systems, as well as the pressure level of the pulmonary artery. Infants typically present with feeding difficulties, failure to thrive, dyspnea, irritability, hyperhidrosis, heart murmurs, tachypnea, tachycardia and/or chest pain while adults usually associate dyspnea, chest pain, syncope, and intolerance to physical exercise. Sudden death may occur due to congestive heart failure, myocardial infarction, valvular insufficiencies or ventricular arrhythmias. The majority of cases reported are of an ALCAPA, while ARCAPA is rarely observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Anomalous origin of coronary artery from the pulmonary artery | ACAPA |
| fr | Anomalie de naissance d'une artère coronaire à partir de l'artère pulmonaire | |
| de | Fehlabgang einer Koronararterie aus der Pulmonalarterie | |
| es | Origen anómalo de la arteria coronaria en la arteria pulmonar | |
| it | Origine anomala dell'arteria coronaria dall'arteria polmonare | |
| nl | Aberrante oorsprong van kransslagader in longslagader | |
| pt | Artéria coronária com origem anómala proveniente da artéria pulmonar | |
| pl | Nieprawidłowe odejście tętnicy wieńcowej od pnia płucnego | = en |
| cs | Anomální odstup koronární tepny z plicnice | = en |
| uk | Аномальне відходження лівої/правої коронарних артерій від стовбура легеневої артерії (ALCAPA, ARCAPA) | = en |
| zh | 肺动脉起源性冠状动脉起源异常 | = en |
| tr | Koroner arterin pulmoner arterden anormal orijini | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q24.5 | NTBT | attributed | validated |
| ICD-11 | LA8C.0 | E | specific | validated |
| MONDO | 0035252 | E | validated | |
| UMLS | C4023252 | 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
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/541507
- This record as JSON: /data/d/541507.json