Heritable pulmonary arterial hypertension
Heritable pulmonary arterial hypertension
Definition
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 familial pulmonary arterial hypertension, FPAH, hereditary pulmonary arterial hypertension, HPAH — per MONDO