{"orpha":431353,"name":"Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis"],"definition":"A disorder that constitutes a rare subgroup of rare pulmonary hypertension characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-431353/","orphanet_url":"https://www.orpha.net/en/disease/detail/431353","names":{"fr":{"name":"Maladie veino-occlusive pulmonaire et/ou hémangiomatose capillaire pulmonaire","definition":"Maladie, qui constitue un sous-groupe d'hypertension pulmonaire rare, caractérisée par une fibrose oblitérante des veines et veinules pulmonaires de petit calibre et/ou une infiltration des capillaires de l'interstitium pulmonaire entraînant une augmentation de la résistance vasculaire pulmonaire menant à la défaillance ventriculaire droite."},"de":{"name":"Pulmonale Venenverschlusskrankheit und/oder pulmonale kapilläre Hämangiomatose","definition":"Die pulmonale venookklusive Erkrankung und/oder die pulmonale kapillare Hämangiomatose (PVOD und/oder PCH) stellt eine seltene Untergruppe der pulmonalen arteriellen Hypertonie (PAH; s. dort) dar, die durch eine obliterative Fibrose der kleinen Lungenvenen und Venolen und/oder eine kapillare Infiltration des pulmonalen Interstitiums gekennzeichnet ist, die zu einem erhöhten pulmonalen Gefäßwiderstand und einer rechtsventrikulären Dysfunktion führt."},"es":{"name":"Enfermedad veno-oclusiva pulmonar y/o hemangiomatosis capilar pulmonar","definition":"Es un trastorno que constituye un subgrupo de hipertensión pulmonar poco frecuente, caracterizada por fibrosis obliterante de las pequeñas vénulas y venas pulmonares y/o infiltración capilar del intersticio pulmonar, que conduce a una mayor resistencia vascular pulmonar y a disfunción ventricular derecha."},"it":{"name":"Malattia veno-occlusiva polmonare e/o emangiomatosi capillare polmonare","definition":"Si tratta di una forma rara di ipertensione arteriosa polmonare, caratterizzata da fibrosi obliterativa delle piccole vene polmonari e delle venule, e/o da infiltrazione capillare dell'interstizio polmonare, che causa un aumento della resistenza vascolare polmonare e una disfunzione del ventricolo destro."},"nl":{"name":"Pulmonale veno-occlusieve ziekte en/of pulmonale capillaire hemangiomatose","definition":"Een stoornis die een zeldzame subgroep vormt van zeldzame pulmonale hypertensie, gekarakteriseerd door obliteratieve fibrose van de kleine longaders en -venulen en/of capillaire infiltratie van het longinterstitium, wat leidt tot een verhoogde vasculaire weerstand in de longen en disfunctie van het rechterventrikel."},"pt":{"name":"Doença veno-oclusiva pulmonar e / ou hemangiomatose capilar pulmonar"},"pl":{"name":"Żylno-okluzyjna choroba płuc i(lub) hemangiomatoza kapilarna płucna","synonyms":["Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis"]},"cs":{"name":"Pulmonální venookluzní choroba a/nebo pulmonální kapilární hemangiomatóza","synonyms":["Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis"]},"uk":{"name":"Легенева венооклюзивна хвороба та/або легеневий капілярний гемангіоматоз","definition":"A disorder that constitutes a rare subgroup of rare pulmonary hypertension characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction."},"zh":{"name":"肺静脉闭塞性疾病和/或肺毛细血管血管瘤病","definition":"包含肺动脉高压的罕见疾病，其特征在于小肺静脉和小静脉闭塞性纤维化和/或肺间质的毛细血管浸润，导致肺血管阻力增加和右心室功能障碍。"},"tr":{"name":"Pulmoner ven-tıkayıcı hastalık ve/veya pulmoner kapiller hemanjiyomatoz"}},"xrefs":[{"source":"MONDO","code":"0018554","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18554","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3698354","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":156610,"name":"Rare genetic respiratory disease","classifications":[156]},{"orpha":71198,"name":"Rare pulmonary hypertension","classifications":[184]}],"children":[{"orpha":199241,"name":"Pulmonary capillary hemangiomatosis","type":"Disease"},{"orpha":31837,"name":"Pulmonary venoocclusive disease","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.015,"area":"France","status":"Validated","source":"20456932[PMID]"}],"phenotypes":null}