{"orpha":182090,"name":"Pulmonary arterial hypertension","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["PAH"],"definition":"Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-182090/","orphanet_url":"https://www.orpha.net/en/disease/detail/182090","names":{"fr":{"name":"Hypertension artérielle pulmonaire","synonyms":["HTAP"],"definition":"Groupe de maladies caractérisées par une résistance artérielle pulmonaire élevée entraînant une insuffisance cardiaque droite. L'hypertension artérielle pulmonaire (HTAP) est progressive et potentiellement létale. Elle peut être idiopathique et/ou familiale, ou secondaire à l'exposition à des médicaments ou à des substances toxiques (HTAP induite par un médicament ou par une substance toxique) ou associée à d'autres maladies, à savoir cardiopathies congénitales, maladies du tissu conjonctif, VIH, schistosomiase, hypertension portale (HTAP associée à d'autres maladies)."},"de":{"name":"Pulmonale arterielle Hypertonie","definition":"Die pulmonal-arterielle Hypertonien (PAH) bilden eine Gruppe von Erkrankungen, die durch einen erhöhten pulmonal-arteriellen Widerstand gekennzeichnet sind, der zu Rechtsherzversagen führt. Die Krankheit ist progressiv und potenziell tödlich. PAH können idiopathisch und/oder familiär bedingt sein oder durch Medikamente oder Toxine induziert werden (medikamenten- oder toxininduzierte PAH, siehe diese Begriffe) oder mit anderen Erkrankungen wie angeborenen Herzerkrankungen, Bindegewebserkrankungen, HIV, Bilharziose, portaler Hypertonie einhergehen (PAH in Verbindung mit anderen Erkrankungen, s. dort)."},"es":{"name":"Hipertensión arterial pulmonar","synonyms":["HAP"],"definition":"Es un grupo de enfermedades caracterizadas por una elevada resistencia arterial pulmonar conducente a una insuficiencia cardíaca derecha. La HAP es progresiva y potencialmente fatal. La HAP puede ser idiopática y/o familiar, o inducida por fármacos o toxinas (HAP inducida por un fármaco o toxina). También puede estar asociada a otras enfermedades, tales como cardiopatía congénita, enfermedad del tejido conectivo, VIH, esquistosomiasis e hipertensión portal (HAP asociada a otras enfermedades)."},"it":{"name":"Ipertensione arteriosa polmonare","synonyms":["PAH"],"definition":"L'ipertensione arteriosa polmonare (PAH) comprende un gruppo di malattie caratterizzate da un'elevata resistenza delle arterie polmonari che esita nell'insufficienza della parte destra del cuore. La malattia è progressiva e potenzialmente letale. La PAH può essere idiopatica e/o familiare, oppure indotta da farmaci o da tossine (PAH indotta dai farmaci o dalle tossine; si vedano questi termini), ma può anche associarsi ad altre malattie, come le cardiopatie congenite, le malattie del tessuto connettivo, l'HIV, la schistosomiasi e l'ipertensione portale (PAH associata ad altra malattia; si veda questo termine)."},"nl":{"name":"Pulmonale arteriële hypertensie","synonyms":["PAH"],"definition":"Pulmonale arteriële hypertensie (PAH) is een groep van ziekten die worden gekarakteriseerd door een verhoogde weerstand in de longslagaders, wat leidt tot een falen van de rechterzijde van het hart. PAH is progressief en potentieel dodelijk. PAH kan idiopathisch en/of familiaal zijn, geïnduceerd worden door een geneesmiddel of een toxine (geneesmiddel- of toxine-geïnduceerde PAH; zie deze termen) of geassocieerd zijn met andere ziekten zoals congenitale hartziekte, bindweefselziekte, HIV, schistosomiase, portale hypertensie (PAH geassocieerd met andere ziekte; zie deze term)."},"pt":{"name":"Hipertensão arterial pulmonar"},"pl":{"name":"Tętnicze nadciśnienie płucne","synonyms":["PAH"]},"cs":{"name":"Plicní arteriální hypertenze","synonyms":["PAH"]},"uk":{"name":"Легенева артеріальна гіпертензія","synonyms":["PAH"],"definition":"Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH, see these terms) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease, see this term)."},"zh":{"name":"肺动脉高压","synonyms":["PAH"],"definition":"肺动脉高压（Pulmonary artery hypertension，PAH）是一组以右心衰竭为特征的疾病，由于肺动脉阻力增高所致，渐进性发展，可能危及生命。肺动脉高压可能是特发性和（或）家族性的，或由药物或毒素引起的（药物或毒素所致肺动脉高压，见术语）或与其他疾病有关，如先天性心脏病、结缔组织疾病、艾滋病、血吸虫病、门脉高压症（肺动脉高压与其他疾病相关，见该术语）。"},"tr":{"name":"Pulmoner arteriyel hipertansiyon","synonyms":["PAH"]}},"xrefs":[{"source":"ICD-11","code":"BB01.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1931148955"},{"source":"MONDO","code":"0015924","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"15924","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000081029","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2973725","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7501","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10064911","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":506222,"name":"Rare disorder potentially indicated for lung transplant","classifications":[233]},{"orpha":71198,"name":"Rare pulmonary hypertension","classifications":[184]}],"children":[{"orpha":275786,"name":"Drug- or toxin-induced pulmonary arterial hypertension","type":"Clinical group"},{"orpha":422,"name":"Idiopathic/heritable pulmonary arterial hypertension","type":"Disease"},{"orpha":275791,"name":"Pulmonary arterial hypertension associated with another disease","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Autosomal dominant","Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]_16456139[PMID]_17360728[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.6,"area":"Spain","status":"Validated","source":"22362843[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.37,"area":"Spain","status":"Validated","source":"22362843[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.5,"area":"France","status":"Validated","source":"16456139[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.24,"area":"France","status":"Validated","source":"16456139[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.09,"area":"United States","status":"Validated","source":"21793646[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.07,"area":"Czech Republic","status":"Validated","source":"24629043[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.24,"area":"Czech Republic","status":"Validated","source":"24629043[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.55,"area":"Switzerland","status":"Validated","source":"18587690[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.35,"area":"Switzerland","status":"Validated","source":"18587690[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.72,"area":"United Kingdom","status":"Validated","source":"21885399[PMID]_17360728[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":5.2,"area":"United Kingdom","status":"Validated","source":"17360728[PMID]"}],"phenotypes":null}