{"orpha":275791,"name":"Pulmonary arterial hypertension associated with another disease","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["PAH associated with another disease","Secondary PAH"],"definition":"Pulmonary arterial hypertension associated with another disease is a group of conditions that lead to PAH; connective tissue diseases (lupus erythematosus, systemic sclerosis and mixed connective tissues disease), congenital heart disease (Eisenmenger syndrome), HIV infection, portal hypertension, schistosomiasis and chronic hemolytic anemia,which is characterized by elevated pulmonary arterial resistance leading to right heart failure that is progressive and potentially fatal.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-275791/","orphanet_url":"https://www.orpha.net/en/disease/detail/275791","names":{"fr":{"name":"Hypertension artérielle pulmonaire associée à une autre maladie","synonyms":["HTAP associée à une autre maladie","HTAP secondaire"],"definition":"Groupe de maladies évoluant vers une HTAP ; maladies du tissu conjonctif (lupus érythémateux, sclérose systémique et connectivite mixte), cardiopathie congénitale (syndrome d'Eisenmenger), infection par le VIH, hypertension portale, schistosomiase et anémie hémolytique chronique, caractérisées par une résistance artérielle pulmonaire élevée conduisant à une insuffisance cardiaque droite progressive et potentiellement létale."},"de":{"name":"Pulmonale arterielle Hypertonie mit assoziierter Krankheit","synonyms":["PAH, sekundäre","PAH mit assoziierter Krankheit"]},"es":{"name":"Hipertensión arterial pulmonar asociada a otra enfermedad","synonyms":["HAP asociada a otra enfermedad","HAP secundaria"],"definition":"Es un grupo de trastornos que derivan en HAP; enfermedades del tejido conjuntivo (lupus eritematoso, esclerosis sistémica y enfermedad mixta del tejido conjuntivo), cardiopatía congénita (síndrome de Eisenmenger), infección por VIH, hipertensión portal, esquistosomiasis y anemia hemolítica crónica. Está caracterizado por una resistencia arterial pulmonar elevada que conduce a insuficiencia cardiaca derecha progresiva y potencialmente fatal."},"it":{"name":"Ipertensione arteriosa polmonare associata ad altra malattia","synonyms":["PAH associata ad altra malattia","PAH secondaria"]},"nl":{"name":"Pulmonale arteriële hypertensie geassocieerd met andere ziekte","synonyms":["PAH geassocieerd met andere ziekte","Secundaire PAH"],"definition":"Pulmonale arteriële hypertensie geassocieerd met andere ziekte is een groep condities die leiden tot PAH (zie deze term), bindweefselziekten (lupus erythematosus, systemische sclerose en gemengde bindweefselziekte), congenitale hartziekte (Eisenmengersyndroom), HIV-infectie, hypertensie in de poortader, schistosomiase en chronische hemolytische anemie (zie deze termen). De groep wordt gekarakteriseerd door een verhoogde weerstand in de longslagaders, wat leidt tot een progressief en potentieel dodelijk falen van de rechterzijde van het hart."},"pt":{"name":"Hipertensão arterial pulmonar associada a outra doença"},"pl":{"name":"Tętnicze nadciśnienie płucne z inną chorobą towarzyszącą","synonyms":["PAH z inną chorobą towarzyszącą","Wtórne PAH"]},"cs":{"name":"Plicní arteriální hypertenze asociovaná s dalším onemocněním","synonyms":["Sekundární PAH","PAH asociovaná s dalším onemocněním"]},"uk":{"name":"Легенева артеріальна гіпертензія, асоційована з іншим захворюванням","synonyms":["ЛАГ, повязана з іншим захворюванням","Вторинна ЛАГ"],"definition":"Pulmonary arterial hypertension associated with another disease is a group of conditions that lead to PAH (see this term); connective tissue diseases (lupus erythematosus, systemic sclerosis and mixed connective tissues disease), congenital heart disease (Eisenmenger syndrome), HIV infection, portal hypertension, schistosomiasis and chronic hemolytic anemia (see these terms),which is characterized by elevated pulmonary arterial resistance leading to right heart failure that is progressive and potentially fatal."},"zh":{"name":"其他疾病相关肺动脉高压","synonyms":["其他疾病相关PAH"],"definition":"与肺动脉高压相关的疾病，即可导致PAH（见该词条)的一组疾病，包括结缔组织疾病(红斑狼疮、系统性硬化症和混合性结缔组织疾病)、先天性心脏病(艾森门格综合征)、HIV感染、门脉高压、血吸虫病和慢性溶血性贫血(见该类词条)，其特征是肺动脉阻力升高导致进行性且可能致命的右心衰竭。"},"tr":{"name":"Başka bir hastalıkla ilişkili pulmoner arteriyel hipertansiyon","synonyms":["Sekonder PAH","Başka bir hastalık ile ilişkili PAH"]}},"xrefs":[{"source":"MONDO","code":"17150","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679756","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":182090,"name":"Pulmonary arterial hypertension","classifications":[184,233]}],"children":[{"orpha":275808,"name":"Pulmonary arterial hypertension associated with HIV infection","type":"Clinical group"},{"orpha":275828,"name":"Pulmonary arterial hypertension associated with chronic hemolytic anemia","type":"Clinical group"},{"orpha":275803,"name":"Pulmonary arterial hypertension associated with congenital heart disease","type":"Clinical group"},{"orpha":275798,"name":"Pulmonary arterial hypertension associated with connective tissue disease","type":"Clinical group"},{"orpha":275813,"name":"Pulmonary arterial hypertension associated with portal hypertension","type":"Clinical group"},{"orpha":275823,"name":"Pulmonary arterial hypertension associated with schistosomiasis","type":"Clinical group"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}