{"orpha":275813,"name":"Pulmonary arterial hypertension associated with portal hypertension","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["PAH associated with portal hypertension","POPH","Portopulmonary hypertension"],"definition":"Pulmonary arterial hypertension associated with portal hypertension (PAH-PH) is a form of pulmonary arterial hypertension (PAH), characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of portal hypertension.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-275813/","orphanet_url":"https://www.orpha.net/en/disease/detail/275813","names":{"fr":{"name":"Hypertension artérielle pulmonaire associée à une hypertension portale","synonyms":["HPoP","HTAP associée à une hypertension portale","Hypertension porto-pulmonaire"],"definition":"Forme d'hypertension artérielle pulmonaire (HTAP) caractérisée par une résistance artérielle pulmonaire élevée entraînant une insuffisance cardiaque droite observée comme complication d'une hypertension portale."},"de":{"name":"Pulmonale arterielle Hypertonie mit assoziierter portaler Hypertension","synonyms":["PAH mit assoziiertem Pfortaderhochdruck","POPH","Portopulmonale Hypertension"]},"es":{"name":"Hipertensión arterial pulmonar asociada a hipertensión portal","synonyms":["HAP asociada a hipertensión portal","Hipertensión portopulmonar"],"definition":"La hipertensión arterial pulmonar asociada a hipertensión portal (HAP-HP) es una forma de hipertensión arterial pulmonar (HAP), caracterizada por una resistencia arterial pulmonar elevada que conduce a insuficiencia cardíaca derecha observada como una complicación de la hipertensión portal."},"it":{"name":"Ipertensione arteriosa polmonare associata a ipertensione portale","synonyms":["Ipertensione portopolmonare","PAH associata a ipertensione portale","POPH"]},"nl":{"name":"Pulmonale arteriële hypertensie geassocieerd met portale hypertensie","synonyms":["PAH geassocieerd met portale hypertensie","POPH","Portopulmonale hypertensie"],"definition":"'Pulmonale arteriële hypertensie geassocieerd met portale hypertensie (PAH-PH) is een vorm van pulmonale arteriële hypertensie (PAH; zie deze term) die wordt gekarakteriseerd door een verhoogde weerstand in de longslagaders die wordt waargenomen als een complicatie van hypertensie in de poortader en die leidt tot een falen van de rechterzijde van het hart.'"},"pt":{"name":"Hipertensão arterial pulmonar associada a hipertensão portal"},"pl":{"name":"Tętnicze nadciśnienie płucne związane z nadciśnieniem wrotnym","synonyms":["Nadciśnienie wrotno-płucne","PAH związane z nadciśnieniem wrotnym","POPH"]},"cs":{"name":"Plicní arteriální hypertenze asociovaná s portální hypertenzí","synonyms":["PAH asociovaná s portální hypertenzí","POPH","Portopulmonální hypertenze"]},"uk":{"name":"Легенева артеріальна гіпертензія, асоційована з портальною гіпертензією","synonyms":["ЛАГ, асоційована з портальною гіпертензією","POPH","Портопульмональна гіпертензія"],"definition":"Pulmonary arterial hypertension associated with portal hypertension (PAH-PH) is a form of pulmonary arterial hypertension (PAH), characterized by an elevated pulmonary arterial resistance leading to right heart failure observed as a complication of portal hypertension."},"zh":{"name":"门脉高压相关肺动脉高压","synonyms":["门脉高压相关PAH"],"definition":"肺动脉高压合并门脉高压(PAH-PH)是肺动脉高压(PAH)的一种，其特征是肺动脉阻力升高导致右心衰竭，被认为是门脉高压的并发症。"},"tr":{"name":"Portal hipertansiyon ilişkili pulmoner arteriyel hipertansiyon","synonyms":["POPH","Portopulmoner hipertansiyon","Portal hipertansiyon ile ilişkili PAH"]}},"xrefs":[{"source":"MONDO","code":"17154","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1868851","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10067281","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":275791,"name":"Pulmonary arterial hypertension associated with another disease","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["No data available"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}