Peripheral pulmonary stenosis
Synonyms: Branch pulmonary artery stenosis · Pulmonary branch stenosis
- Type
- Morphological anomaly
- Group
- Disorder
- Status
- active
Peripheral pulmonary stenosis is a rare congenital anomaly of the great arteries that may occur at single or multiple sites, in isolation or in association with other congenital heart defects (valvular pulmonary stenosis, atrial, or ventricular septal defects or tetralogy of Fallot) and genetic syndromes (Williams, Alagile syndrome). Clinical presentation is variable and includes heart murmurs, dyspnea, syncope, chest pain and pulmonary hypertension-associated symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Peripheral pulmonary stenosis | Branch pulmonary artery stenosis · Pulmonary branch stenosis |
| fr | Sténoses des branches pulmonaires | Sténose de l'artère pulmonaire · Sténose des branches de l'artère pulmonaire |
| de | Periphere Pulmonalstenose | Pulmonalisaststenose · Stenose der Pulmonalarterienäste |
| es | Estenosis pulmonar periférica | Estenosis de la rama de la arteria pulmonar · Estenosis de ramas pulmonares |
| it | Stenosi polmonare periferica | |
| nl | Perifere stenose van arteria pulmonalis | Perifere stenose van longslagader · Pulmonaaltakstenose · Stenose van tak van arteria pulmonalis · Stenose van tak van longslagader |
| pt | Estenose dos ramos pulmonares | |
| pl | Zwężenie gałęzi tętnicy płucnej | = en |
| cs | Stenóza větve plicnice | = en |
| uk | Периферичний стеноз легеневої артерії | Стеноз гілки легеневої артерії · Стеноз легеневої гілки |
| zh | 周围肺动脉狭窄 | 分支性肺动脉狭窄 |
| tr | Periferik pulmoner stenoz | Pulmoner dal darlığı · Dal pulmoner arter darlığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | Q25.6 | E | specific | validated |
| ICD-11 | LA8B.1 | BTNT | specific | validated |
| MONDO | 0020420 | E | validated | |
| UMLS | C3531782 | E | validated | |
| GARD | 4589 | 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/99084
- This record as JSON: /data/d/99084.json