{"orpha":541454,"name":"Anomalous aortic origin of the right coronary artery","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["AORCA","R-ACAOS","Right coronary artery from left aortic sinus"],"definition":"A rare coronary artery congenital malformation characterized by an anomalous origin and course of the right coronary artery, which originates from the left aortic sinus of Valsalva and has an abnormal proximal course, which may be intramural, prepulmonic, subpulmonic, retroaortic, retrocardiac or wrapped around the apex. Patients are frequently asymptomatic, although chest pain, dyspnea, palpitations, dizziness, syncope, and sudden cardiac arrest/death (typically following intense physical exertion) may be observed. This malformation is associated with a lower risk of sudden cardiac death therefore surgical revascularization is recommended only when signs and/or symptoms of ischemia are present.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-541454/","orphanet_url":"https://www.orpha.net/en/disease/detail/541454","names":{"fr":{"name":"Anomalie de naissance de l'artère coronaire droite à partir de l'aorte","synonyms":["Anomalie d'origine de l'artère coronaire droite à partir de l'aorte","Naissance de l'artère coronaire droite à partir du sinus gauche","R-ACAOS"],"definition":"Anomalie congénitale rare des artères coronaires caractérisée par une anomalie de naissance et de trajet de l'artère coronaire droite, à partir du sinus de Valsalva gauche et dont le trajet proximal est anormal ; ce segment de l'artère peut en effet être intramural, prépulmonaire, sous-pulmonaire, rétro-aortique, rétrocardiaque ou enroulé tout autour de l'apex. Les patients sont souvent asymptomatiques, bien que douleurs thoraciques, dyspnée, palpitations, vertiges, syncopes et arrêt cardiaque/décès (généralement après un effort intense) puissent être observés. Cette malformation étant associée à un plus faible risque d'arrêt cardiorespiratoire, une revascularisation chirurgicale n'est recommandée qu'en présence de signes et/ou de symptômes d'ischémie."},"de":{"name":"Anomaler Abgang der rechten Koronararterie","synonyms":["AORCA","Fehlabgang der rechten Koronararterie aus dem linken Aortensinus","R-ACAOS"],"definition":"Eine seltene angeborene Koronararterienfehlbildung, die durch einen anomalen Ursprung und Verlauf der rechten Koronararterie gekennzeichnet ist, die aus dem linken Aortensinus entspringt und einen anomalen proximalen Verlauf hat, der intramural, präpulmonal, subpulmonal, retroaortal, retrokardial oder um den Apex gewickelt sein kann. Die Patienten sind häufig asymptomatisch, obwohl Brustschmerzen, Dyspnoe, Palpitationen, Schwindel, Synkopen und plötzlicher Herzstillstand/Tod (typischerweise nach intensiver körperlicher Anstrengung) beobachtet werden können. Diese Fehlbildung ist mit einem geringeren Risiko für einen plötzlichen Herztod assoziiert, daher wird eine chirurgische Revaskularisation nur dann empfohlen, wenn Zeichen und/oder Symptome einer Ischämie vorliegen."},"es":{"name":"Origen anómalo de la arteria coronaria derecha","synonyms":["AORCA","Arteria coronaria derecha en el seno aórtico izquierdo","R-ACAOS"],"definition":"Es una malformación congénita poco frecuente de las arterias coronarias caracterizada por un origen y un curso anómalo de la arteria coronaria derecha, que se origina en el seno de Valsalva izquierdo y presenta un curso proximal anómalo, pudiendo ser intramural, prepulmonar, subpulmonar, retroaórtico, retrocardíaco o rodeando el apex. Con frecuencia, los pacientes permanecen asintomáticos, aunque puede observarse dolor torácico, disnea, palpitaciones, mareos, síncope y paro cardíaco/ muerte súbita (por lo general, después de un esfuerzo físico intenso). Esta malformación se asocia a un menor riesgo de muerte súbita cardíaca; por lo tanto, la revascularización quirúrgica sólo está recomendada cuando hay signos y/o síntomas de isquemia."},"it":{"name":"Origine anomala dell'arteria coronaria destra dall'aorta","definition":"Si tratta di una malformazione congenita rara dell'arteria coronaria, caratterizzata da anomalie dell'origine e del decorso dell'arteria coronaria destra, che origina dal seno aortico di Valsalva sinistro e ha uno sviluppo prossimale anomalo, che può essere intramurale, prepolmonare, sottopolmonare, retroaortico, retrocardiaco o avvolto attorno all'apice. Spesso i pazienti sono asintomatici, ma possono sviluppare a ogni età dolore toracico, dispnea, palpitazioni, vertigini, sincope e arresto/morte cardiaca improvvisa (in genere dopo un esercizio fisico intenso). Questa malformazione si associa a un rischio inferiore di morte cardiaca improvvisa; perciò, la rivascolarizzazione chirurgica è indicata solo in presenza di segni clinici e/o sintomi dell'ischemia."},"nl":{"name":"Aberrante oorsprong van rechterkransslagader in aorta","synonyms":["AORCA","R-ACAOS","Rechterkransslagader uit linker sinus van Valsalva"],"definition":"Een zeldzame, congenitale malformatie van de kransslagaders, gekarakteriseerd door een aberrante oorsprong en een aberrant verloop van de rechterkransslagader, die ontspringt uit de linker sinus van Valsalva en die een abnormaal proximaal verloop heeft dat intramuraal, prepulmonaal, subpulmonaal, retroaortaal, retrocardiaal of gewikkeld rond de apex kan zijn. Patiënten zijn vaak asymptomatisch, hoewel pijn in de borst, dyspneu, hartkloppingen, duizeligheid, syncope, en plotse hartstilstand/dood (doorgaans na intense fysieke inspanning) waargenomen kunnen worden. Deze malformatie is geassocieerd met een lager risico op plotse hartdood, en bijgevolg wordt chirurgische revascularisatie enkel aanbevolen wanneer tekenen en/of symptomen van ischemie aanwezig zijn."},"pt":{"name":"Artéria coronária direita com origem aórtica anómala"},"pl":{"name":"Nieprawidłowe odejście prawej tętnicy wieńcowej od aorty","synonyms":["AORCA","R-ACAOS","Right coronary artery from left aortic sinus"]},"cs":{"name":"Anomální aortální odstup pravé koronární tepny","synonyms":["AORCA","R-ACAOS","Pravá koronární tepna z levého aortálního sinu"]},"uk":{"name":"Аномальне відходження правої коронарної артерії від аорти","synonyms":["AORCA","R-ACAOS","Права коронарна артерія від лівого аортального синуса"],"definition":"A rare coronary artery congenital malformation characterized by an anomalous origin and course of the right coronary artery, which originates from the left aortic sinus of Valsalva and has an abnormal proximal course, which may be intramural, prepulmonic, subpulmonic, retroaortic, retrocardiac or wrapped around the apex. Patients are frequently asymptomatic, although chest pain, dyspnea, palpitations, dizziness, syncope, and sudden cardiac arrest/death (typically following intense physical exertion) may be observed. This malformation is associated with a lower risk of sudden cardiac death therefore surgical revascularization is recommended only when signs and/or symptoms of ischemia are present."},"zh":{"name":"主动脉起源性右冠状动脉起源异常","synonyms":["右冠状动脉起源于左主动脉窦"],"definition":"一种罕见的冠状动脉先天性畸形，其特征是右冠状动脉起源和走向异常，右冠状动脉起源于Valsalva左主动脉窦，近端走向异常，可为腔内、腔前、肺下、主动脉后、心后或包绕心尖。患者常无症状，但可观察到胸痛、呼吸困难、心悸、头晕、晕厥和心脏骤停/死亡（通常在剧烈体力消耗后）。这种畸形与较低风险的心脏性猝死相关，因此只有当出现缺血的症状和/或体征时，才建议进行血管重建手术。"},"tr":{"name":"Sağ koroner arterin anormal aort orijini","synonyms":["AORCA","R-ACAOS","Sol aort sinüsünden sağ koroner arter"]}},"xrefs":[{"source":"ICD-10","code":"Q24.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA8C.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"912125792"},{"source":"MONDO","code":"0035250","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3532025","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":541478,"name":"Anomalous aortic origin of coronary artery","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}