Pulmonary arterial hypertension associated with portal hypertension
Synonyms: PAH associated with portal hypertension · POPH · Portopulmonary hypertension
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- No data available
- Age of onset
- Adult
- Status
- active
Pulmonary arterial hypertension associated with portal hypertension (PAH-PH) is a form of pulmonary arterial hypertension (PAH), characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of portal hypertension.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary arterial hypertension associated with portal hypertension | PAH associated with portal hypertension · POPH · Portopulmonary hypertension |
| fr | Hypertension artérielle pulmonaire associée à une hypertension portale | HPoP · HTAP associée à une hypertension portale · Hypertension porto-pulmonaire |
| de | Pulmonale arterielle Hypertonie mit assoziierter portaler Hypertension | PAH mit assoziiertem Pfortaderhochdruck · POPH · Portopulmonale Hypertension |
| es | Hipertensión arterial pulmonar asociada a hipertensión portal | HAP asociada a hipertensión portal · Hipertensión portopulmonar |
| it | Ipertensione arteriosa polmonare associata a ipertensione portale | Ipertensione portopolmonare · PAH associata a ipertensione portale · POPH |
| nl | Pulmonale arteriële hypertensie geassocieerd met portale hypertensie | PAH geassocieerd met portale hypertensie · POPH · Portopulmonale hypertensie |
| pt | Hipertensão arterial pulmonar associada a hipertensão portal | |
| pl | Tętnicze nadciśnienie płucne związane z nadciśnieniem wrotnym | Nadciśnienie wrotno-płucne · PAH związane z nadciśnieniem wrotnym · POPH |
| cs | Plicní arteriální hypertenze asociovaná s portální hypertenzí | PAH asociovaná s portální hypertenzí · POPH · Portopulmonální hypertenze |
| uk | Легенева артеріальна гіпертензія, асоційована з портальною гіпертензією | ЛАГ, асоційована з портальною гіпертензією · POPH · Портопульмональна гіпертензія |
| zh | 门脉高压相关肺动脉高压 | 门脉高压相关PAH |
| tr | Portal hipertansiyon ilişkili pulmoner arteriyel hipertansiyon | POPH · Portopulmoner hipertansiyon · Portal hipertansiyon ile ilişkili PAH |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 17154 | E | validated | |
| UMLS | C1868851 | E | validated | |
| MedDRA | 10067281 | 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:275791Pulmonary arterial hypertension associated with another disease
rare respiratory diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
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/275813
- This record as JSON: /data/d/275813.json