Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition Heritable pulmonary arterial hypertension (HPAH) is a form of pulmonary arterial hypertension (PAH, see this term), 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. Disease data Classification Etiological subtype Synonyms FPAH FPAH Hereditary pulmonary arterial hypertension Rodzinne tętnicze nadciśnienie płucne Familial pulmonary arterial hypertension HPAH Hereditary pulmonary arterial hypertension ORPHA code 275777 OMIM code 178600 ICD10 code I27.0 ICD11 code BB01.0 *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl