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Idiopathic/heritable pulmonary arterial hypertension
A form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure; it is progressive and potentially fatal. The majority cases have an identifiable genetic cause, but a significant proportion are idiopathic.
ORPHA:422
Classification level: Disorder
- Idiopathic and/or familial pulmonary arterial hypertension
Prevalence: 1-9 / 100 000
Inheritance: Autosomal dominant, Autosomal recessive, Not applicable
Age of onset: All ages
: produced/endorsed by ERN(s)
: produced/endorsed by FSMR(s)
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