Idiopathic pulmonary artery dilatation
- Type
- Disease
- Group
- Disorder
- Status
- active
Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery, with or without dilatation of the right and left pulmonary artery branches, and not attributed to any other cardiac, pulmonary and/or arterial wall disease. It may present with exertional dyspnea, fatigue, cough, hemoptysis, palpitation and chest pain, but may also be asymptomatic. In serious cases, trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic pulmonary artery dilatation | |
| fr | Dilatation idiopathique de l'artère pulmonaire | |
| de | Pulmonalarteriendilatation, idiopathische | |
| es | Dilatación idiopática de la arteria pulmonar | |
| it | Dilatazione idiopatica dell'arteria polmonare | |
| nl | Idiopathische pulmonale arteriële dilatatie | |
| pt | Dilatação da artéria pulmonar familiar | |
| pl | Idiopatyczne rozszerzenie tętnicy płucnej | |
| cs | Idiopatická plicní arteriální dilatace | |
| uk | Ідіопатичне розширення легеневої артерії | |
| zh | 特发性肺动脉扩张 | |
| tr | İdiyopatik pulmoner arter dilatasyonu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I28.8 | NTBT | attributed | validated |
| ICD-11 | LA8B.1 | NTBT | index | validated |
| MONDO | 0015664 | E | validated | |
| UMLS | C1384816 | E | validated | |
| GARD | 6757 | 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:98719Pulmonary artery or pulmonary branch anomaly
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/1676
- This record as JSON: /data/d/1676.json