Pulmonary arterial hypertension associated with another disease
Synonyms: PAH associated with another disease · Secondary PAH
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Pulmonary arterial hypertension associated with another disease is a group of conditions that lead to PAH; connective tissue diseases (lupus erythematosus, systemic sclerosis and mixed connective tissues disease), congenital heart disease (Eisenmenger syndrome), HIV infection, portal hypertension, schistosomiasis and chronic hemolytic anemia,which is characterized by elevated pulmonary arterial resistance leading to right heart failure that is progressive and potentially fatal.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary arterial hypertension associated with another disease | PAH associated with another disease · Secondary PAH |
| fr | Hypertension artérielle pulmonaire associée à une autre maladie | HTAP associée à une autre maladie · HTAP secondaire |
| de | Pulmonale arterielle Hypertonie mit assoziierter Krankheit | PAH, sekundäre · PAH mit assoziierter Krankheit |
| es | Hipertensión arterial pulmonar asociada a otra enfermedad | HAP asociada a otra enfermedad · HAP secundaria |
| it | Ipertensione arteriosa polmonare associata ad altra malattia | PAH associata ad altra malattia · PAH secondaria |
| nl | Pulmonale arteriële hypertensie geassocieerd met andere ziekte | PAH geassocieerd met andere ziekte · Secundaire PAH |
| pt | Hipertensão arterial pulmonar associada a outra doença | |
| pl | Tętnicze nadciśnienie płucne z inną chorobą towarzyszącą | PAH z inną chorobą towarzyszącą · Wtórne PAH |
| cs | Plicní arteriální hypertenze asociovaná s dalším onemocněním | Sekundární PAH · PAH asociovaná s dalším onemocněním |
| uk | Легенева артеріальна гіпертензія, асоційована з іншим захворюванням | ЛАГ, повязана з іншим захворюванням · Вторинна ЛАГ |
| zh | 其他疾病相关肺动脉高压 | 其他疾病相关PAH |
| tr | Başka bir hastalıkla ilişkili pulmoner arteriyel hipertansiyon | Sekonder PAH · Başka bir hastalık ile ilişkili PAH |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 17150 | E | validated | |
| UMLS | C5679756 | 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:182090Pulmonary arterial hypertension
rare respiratory diseases; rare transplant-related disorders
Children (6):
- ORPHA:275808Pulmonary arterial hypertension associated with HIV infection
- ORPHA:275828Pulmonary arterial hypertension associated with chronic hemolytic anemia
- ORPHA:275803Pulmonary arterial hypertension associated with congenital heart disease
- ORPHA:275798Pulmonary arterial hypertension associated with connective tissue disease
- ORPHA:275813Pulmonary arterial hypertension associated with portal hypertension
- ORPHA:275823Pulmonary arterial hypertension associated with schistosomiasis
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/275791
- This record as JSON: /data/d/275791.json