{"orpha":1676,"name":"Idiopathic pulmonary artery dilatation","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery, with or without dilatation of the right and left pulmonary artery branches, and not attributed to any other cardiac, pulmonary and/or arterial wall disease. It may present with exertional dyspnea, fatigue, cough, hemoptysis, palpitation and chest pain, but may also be asymptomatic. In serious cases, trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1676/","orphanet_url":"https://www.orpha.net/en/disease/detail/1676","names":{"fr":{"name":"Dilatation idiopathique de l'artère pulmonaire","definition":"La dilatation idiopathique de l'artère pulmonaire est une anomalie rare du développement survenant au cours de l'embryogenèse. Elle se caractérise par la dilatation de l'artère pulmonaire principale, avec ou sans dilatation des branches artérielles pulmonaires droites et gauches, en absence d'un autre trouble cardiaque, pulmonaire et/ou de la paroi artérielle. Elle peut se manifester par une dyspnée à l'effort, une fatigue, une toux, une hémoptysie, des palpitations et une douleur thoracique, mais elle peut aussi être asymptomatique. Dans les cas sévères, la constriction de la trachée due aux changements de posture peut conduire à des crises cyanotiques avec dyspnée sévère. Une mort subite d'origine cardiaque a été rapportée dans certains cas."},"de":{"name":"Pulmonalarteriendilatation, idiopathische","definition":"Die idiopathische Lungenarteriendilatation ist ein seltener Entwicklungsfehler während der Embryogenese, der durch die Dilatation der Hauptlungenarterie mit oder ohne Dilatation der rechten und linken Lungenarterienäste gekennzeichnet ist und nicht auf eine andere kardiale, pulmonale und/oder arterielle Wanderkrankung zurückzuführen ist. Sie kann sich mit Belastungsdyspnoe, Müdigkeit, Husten, Hämoptysen, Herzklopfen und Brustschmerzen äußern, kann aber auch asymptomatisch sein. In schweren Fällen kann eine Verengung der Luftröhre aufgrund von Haltungsänderungen zu Anfällen von Zyanose mit schwerer Dyspnoe führen. In einigen Fällen wurde über plötzlichen Herztod berichtet."},"es":{"name":"Dilatación idiopática de la arteria pulmonar","definition":"La dilatación idiopática de la arteria pulmonar es un defecto del desarrollo durante la embriogénesis poco frecuente que se caracteriza por la dilatación de la arteria pulmonar principal, con o sin dilatación de las ramas derecha e izquierda, y no atribuida a ninguna otra enfermedad cardíaca, pulmonar y/o de la pared arterial. Puede presentarse con disnea de esfuerzo, fatiga, tos, hemoptisis, palpitaciones y dolor en el pecho, pero también puede ser asintomática. En casos graves, la constricción de la tráquea debido a cambios posturales puede provocar ataques de cianosis con grave disnea. En algunos casos, se ha informado de muerte súbita cardíaca."},"it":{"name":"Dilatazione idiopatica dell'arteria polmonare","definition":"La dilatazione idiopatica dell'arteria polmonare è un difetto raro dello sviluppo dell'embrione, caratterizzato dalla dilatazione dell'arteria polmonare principale, con o senza dilatazione dei rami destro e sinistro dell'arteria polmonare, non causata da altre malattie cardiache, polmonari e/o della parete arteriosa. Esordisce con dispnea da sforzo, affaticamento, tosse, emottisi, palpitazioni e dolore toracico, ma può anche essere asintomatica. Nei casi più gravi, la costrizione della trachea, secondaria alle modifiche della postura, può causare attacchi di cianosi associati a dispnea grave. Sono stati descritti casi di morte cardiaca improvvisa."},"nl":{"name":"Idiopathische pulmonale arteriële dilatatie","definition":"Idiopathische pulmonale arteriële dilatatie is een zeldzaam ontwikkelingsdefect tijdens de embryogenese dat gekarakteriseerd wordt door dilatatie van de longslagader, met of zonder dilatatie van de rechter en linker aftakkingen van de longslagader, en dat niet toe te schrijven is aan enige andere ziekte van het hart, de longen of de slagaderwanden. De ziekte kan zich uiten met inspanningsafhankelijke dyspneu, vermoeidheid, hoest, hemoptoë, hartkloppingen (palpitaties) en pijn in de borst, maar kan ook asymptomatisch zijn. In ernstige gevallen kan constrictie van de luchtpijp als gevolg van veranderingen in lichaamshouding leiden tot aanvallen van cyanose met ernstige dyspneu. In sommige gevallen werd acute hartdood gerapporteerd."},"pt":{"name":"Dilatação da artéria pulmonar familiar"},"pl":{"name":"Idiopatyczne rozszerzenie tętnicy płucnej"},"cs":{"name":"Idiopatická plicní arteriální dilatace"},"uk":{"name":"Ідіопатичне розширення легеневої артерії","definition":"Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery, with or without dilatation of the right and left pulmonary artery branches, and not attributed to any other cardiac, pulmonary and/or arterial wall disease. It may present with exertional dyspnea, fatigue, cough, hemoptysis, palpitation and chest pain, but may also be asymptomatic. In serious cases, trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases."},"zh":{"name":"特发性肺动脉扩张"},"tr":{"name":"İdiyopatik pulmoner arter dilatasyonu"}},"xrefs":[{"source":"ICD-10","code":"I28.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA8B.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2022819457"},{"source":"MONDO","code":"0015664","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1384816","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6757","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":98719,"name":"Pulmonary artery or pulmonary branch anomaly","classifications":[147,148,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}