Pulmonary arterial hypertension
ORPHA:182090
Definition
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).
- Prevalence
- 1-9 / 100 000
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- All ages