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Heritable pulmonary arterial hypertension

rare

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.

Identifiers
MONDO:0017148 Orphanet:275777
Part of
Hereditary diseasePulmonary arterial hypertension
Also known as
familial pulmonary arterial hypertension · FPAH · hereditary pulmonary arterial hypertension · HPAH

Subtypes

All diseases Rare only
Disease Subtypes
Pulmonary hypertension, primary, 1
Pulmonary hypertension, primary, 2
Pulmonary hypertension, primary, 3
Pulmonary hypertension, primary, 4
Pulmonary hypertension, primary, 5
Pulmonary hypertension, primary, 6
Pulmonary hypertension, primary, 7

Rare disease

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