Pulmonary arterial hypertension
Synonyms: PAH
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- All ages
- Status
- active
Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary arterial hypertension | PAH |
| fr | Hypertension artérielle pulmonaire | HTAP |
| de | Pulmonale arterielle Hypertonie | |
| es | Hipertensión arterial pulmonar | HAP |
| it | Ipertensione arteriosa polmonare | = en |
| nl | Pulmonale arteriële hypertensie | = en |
| pt | Hipertensão arterial pulmonar | |
| pl | Tętnicze nadciśnienie płucne | = en |
| cs | Plicní arteriální hypertenze | = en |
| uk | Легенева артеріальна гіпертензія | = en |
| zh | 肺动脉高压 | = en |
| tr | Pulmoner arteriyel hipertansiyon | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | BB01.0 | E | specific | validated |
| MONDO | 0015924 | E | validated | |
| MONDO | 15924 | E | validated | |
| MeSH | D000081029 | E | validated | |
| UMLS | C2973725 | E | validated | |
| GARD | 7501 | E | validated | |
| MedDRA | 10064911 | 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 (2):
- ORPHA:506222Rare disorder potentially indicated for lung transplant
rare transplant-related disorders - ORPHA:71198Rare pulmonary hypertension
rare respiratory diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
12 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2018[OTHER]_16456139[PMID]_17360728[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.6 / 100 000 | Spain | Value and class | Validated | 22362843[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.37 / 100 000 | Spain | Value and class | Validated | 22362843[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.5 / 100 000 | France | Value and class | Validated | 16456139[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.24 / 100 000 | France | Value and class | Validated | 16456139[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.09 / 100 000 | United States | Value and class | Validated | 21793646[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.07 / 100 000 | Czech Republic | Value and class | Validated | 24629043[PMID] |
| Point prevalence | 1-9 / 100 000 | 2.24 / 100 000 | Czech Republic | Value and class | Validated | 24629043[PMID] |
| Point prevalence | 1-9 / 100 000 | 1.55 / 100 000 | Switzerland | Value and class | Validated | 18587690[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.35 / 100 000 | Switzerland | Value and class | Validated | 18587690[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.72 / 100 000 | United Kingdom | Value and class | Validated | 21885399[PMID]_17360728[PMID] |
| Point prevalence | 1-9 / 100 000 | 5.2 / 100 000 | United Kingdom | Value and class | Validated | 17360728[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/182090
- This record as JSON: /data/d/182090.json