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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