Heritable pulmonary arterial hypertension

MONDO:0017148

Heritable pulmonary arterial hypertension (HPAH) is a form of pulmonary arterial hypertension (PAH), occurring due to mutations in PAH predisposing genes or in a familial context. HPAH is characterized by elevated pulmonary arterial resistance leading to right heart failure. HPAH is progressive and potentially fatal.

Also known as: FPAH, HPAH, HpaH, familial pulmonary arterial hypertension, hereditary pulmonary arterial hypertension

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