{"orpha":642071,"name":"Primary pulmonary vein stenosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Congenital pulmonary vein stenosis","PPVS"],"definition":null,"note":null,"url":"https://raremap.alftech.space/d/ORPHA-642071/","orphanet_url":"https://www.orpha.net/en/disease/detail/642071","names":{"fr":{"name":"Sténose primaire des veines pulmonaires","synonyms":["Sténose congénitale des veines pulmonaires"]},"de":{"name":"Pulmonalvenenstenose, primäre","synonyms":["PPVS"]},"es":{"name":"Estenosis de vena pulmonar primaria","synonyms":["PPVS"]},"it":{"name":"Stenosi primaria delle vene polmonari","synonyms":["PPVS"]},"nl":{"name":"Primaire stenose van vena pulmonalis","synonyms":["PPVS","Primaire stenose van longader"]},"pt":{"name":"Estenose primária da veia pulmonar","synonyms":["PPVS"]},"pl":{"name":"Pierwotne zwężenie żył płucnych","synonyms":["Congenital pulmonary vein stenosis","PPVS"]},"cs":{"name":"Primární stenóza plicní žíly","synonyms":["Congenital pulmonary vein stenosis","PPVS"]},"uk":{"name":"Первинний стеноз легеневої вени","synonyms":["Вроджений стеноз легеневої вени","PPVS"]}},"xrefs":[{"source":"ICD-10","code":"Q26.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA86.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"469101490"},{"source":"MONDO","code":"0957433","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0265915","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":3188,"name":"Congenital pulmonary veins atresia or stenosis","classifications":[147,148,184,198,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}