Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition Idiopathic pulmonary arterial hypertension (IPAH) is a sporadic form of pulmonary arterial hypertension (PAH, see this term) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH. Disease data Classification Etiological subtype Synonyms IPAH IPAH Pierwotne tętnicze nadciśnienie płucne Primary pulmonary arterial hypertension ORPHA code 275766 OMIM code - 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