{"orpha":99075,"name":"Encircling double aortic arch","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Encircling double aortic arch is a very rare congenital anomaly of the great arteries characterized by the presence of two aortic arches (right and left) which encircle and compress the trachea and esophagus, resulting in various respiratory and gastrointestinal symptoms (e.g. harsh breathing, stridor, dyspnea, cyanotic and choking episodes, chronic cough, recurrent respiratory tract infections, dysphagia and reflux). Esophageal atresia and tracheoesophageal fistula have also been reported. It usually occurs isolated, but, on occasion, may be associated with other congenital heart anomalies and chromosomal aberations.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99075/","orphanet_url":"https://www.orpha.net/en/disease/detail/99075","names":{"fr":{"name":"Aorte encerclante","definition":"Une anomalie congénitale très rare des gros vaisseaux. Elle se caractérise par la présence de deux arcs aortiques (droite et gauche) qui encerclent et compriment la trachée et l'oesophage, conduisant à divers signes respiratoires et gastro-intestinaux (comme une respiration difficile, un stridor, une dyspnée, des épisodes cyanotiques et d'état de choc, une toux chronique, des infections récurrentes des voies respiratoires, une dysphagie et un reflux). Une atrésie oesophagienne et une fistule trachéo-oesophagienne ont aussi été rapportées. L'anomalie survient généralement de manière isolée, mais parfois, elle peut être associée à d'autres anomalies cardiaques congénitales et à des aberrations chromosomiques."},"de":{"name":"Doppelter Aortenbogen mit zirkulärer Einengung von Trachea/Ösophagus","definition":"Eine sehr seltene angeborene Anomalie der großen Arterien, die durch das Vorhandensein von zwei Aortenbögen (rechts und links) gekennzeichnet ist, die die Luftröhre und den Ösophagus umschließen und komprimieren, was zu verschiedenen respiratorischen und gastrointestinalen Symptomen führt (z. B. erschwerte Atmung, Stridor, Dyspnoe, zyanotische und würgende Episoden, chronischer Husten, rezidivierende Atemwegsinfektionen, Dysphagie und Reflux). Ösophagusatresie und tracheoösophageale Fistel sind ebenfalls berichtet worden. Die Fehlbildung tritt in der Regel isoliert auf, kann aber gelegentlich mit anderen kongenitalen Herzanomalien und Chromosomenaberrationen assoziiert sein."},"es":{"name":"Anillo vascular por doble arco aórtico","definition":"El anillo vascular por doble arco aórtico es una anomalía congénita muy poco frecuente de las grandes arterias caracterizada por la presencia de dos arcos aórticos (derecho e izquierdo) que rodean y comprimen la tráquea y el esófago, lo que produce diversos síntomas respiratorios y gastrointestinales (p. ej., respiración áspera, estridor, disnea, episodios cianóticos y de asfixia, tos crónica, infecciones recurrentes del tracto respiratorio, disfagia y reflujo). También se ha notificado atresia esofágica y fístula traqueoesofágica. Por lo general, se presenta aislado, pero en ocasiones, puede estar asociado a otras anomalías cardíacas congénitas y anomalías cromosómicas."},"it":{"name":"Doppio arco aortico","definition":"Il doppio arco aortico è un'anomalia congenita molto rara delle grandi arterie, caratterizzata dalla presenza di due archi aortici (destro e sinistro) che accerchiano e comprimono la trachea e l'esofago, causando sintomi respiratori e gastrointestinali (difficoltà respiratorie, stridore, dispnea, episodi di cianosi e soffocamento, tosse cronica, infezioni ricorrenti delle vie aeree, disfagia e reflusso). Sono stati descritti pazienti con atresia esofagea e fistola tracheoesofagea. Di solito la malformazione è isolata, ma può associarsi occasionalmente ad altre cardiopatie congenite e ad aberrazioni cromosomiche."},"nl":{"name":"Omcirkelende dubbele aortaboog","definition":"Omcirkelende dubbele aortaboog is een zeer zeldzame congenitale anomalie van de grote arteriën die gekarakteriseerd wordt door de aanwezigheid van twee aortabogen (rechts en links) die de luchtpijp en de slokdarm omcirkelen en samendrukken, wat leidt tot verscheidene respiratoire en gastro-intestinale symptomen (e.g. hard ademen, stridor, dyspneu, episoden van cyanose en verstikking, chronische hoest, terugkerende infecties van de luchtwegen, dysfagie en reflux). Oesofageale atresie en tracheo-oesofageale fistel werden ook reeds gerapporteerd. Meestal komt de aandoening geïsoleerd voor, maar soms kan het geassocieerd zijn met andere congenitale hartanomalieën en chromosomale afwijkingen."},"pt":{"name":"Arco aórtico duplo"},"pl":{"name":"Podwójny łuk aorty"},"cs":{"name":"Dvojitý aortální oblouk"},"uk":{"name":"Огинаюча подвійна дуга аорти","definition":"Encircling double aortic arch is a very rare congenital anomaly of the great arteries characterized by the presence of two aortic arches (right and left) which encircle and compress the trachea and esophagus, resulting in various respiratory and gastrointestinal symptoms (e.g. harsh breathing, stridor, dyspnea, cyanotic and choking episodes, chronic cough, recurrent respiratory tract infections, dysphagia and reflux). Esophageal atresia and tracheoesophageal fistula have also been reported. It usually occurs isolated, but, on occasion, may be associated with other congenital heart anomalies and chromosomal aberations."},"zh":{"name":"围绕型双主动脉弓","definition":"围绕型双主动脉弓是一种非常罕见的先天性大动脉畸形，其特征是左右两个主动脉弓环绕并压迫气管和食道，导致各种呼吸道和胃肠道症状(如呼吸急促、气喘吁吁、呼吸困难、紫绀和窒息、慢性咳嗽、反复呼吸道感染、吞咽困难和反流)，食道闭锁和气管食管瘘也有报道。它通常是孤立发生的，但有时也可能与其他先天性心脏畸形和染色体异常有关。"},"tr":{"name":"Çevreleyen çift aortik ark"}},"xrefs":[{"source":"ICD-10","code":"Q25.4","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"LA8B.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"960436403"},{"source":"MONDO","code":"0020413","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4706940","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":1132,"name":"Aortic arch defects","classifications":[147,148,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}