{"orpha":541443,"name":"Anomalous aortic origin of the left coronary artery","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["AOLCA","L-ACAOS","Left coronary artery from right aortic sinus"],"definition":"A rare coronary artery congenital malformation characterized by an anomalous origin and course of the left coronary artery, which originates from the right 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 high risk of sudden cardiac death so surgical revascularization is recommended even in cases with no associated evidence of myocardial ischemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-541443/","orphanet_url":"https://www.orpha.net/en/disease/detail/541443","names":{"fr":{"name":"Anomalie de naissance de l'artère coronaire gauche à partir de l'aorte","synonyms":["Anomalie d'origine de l'artère coronaire gauche à partir de l'aorte","L-ACAOS","Naissance de l'artère coronaire gauche à partir du sinus droit"],"definition":"Anomalie congénitale rare des artères coronaires caractérisée par une anomalie de naissance et de trajet de l'artère coronaire gauche, à partir du sinus de Valsalva droit 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 risque élevé d'arrêt cardiorespiratoire, une revascularisation chirurgicale est recommandée même en l'absence de signes d'ischémie myocardique."},"de":{"name":"Anomaler Abgang der linken Koronararterie","synonyms":["Fehlabgang der linken Koronararterie aus dem rechten Aortensinus"],"definition":"Eine seltene angeborene Koronararterienfehlbildung, die durch einen anomalen Ursprung und Verlauf der linken Koronararterie gekennzeichnet ist, die aus dem rechten 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 hohen Risiko für einen plötzlichen Herztod verbunden, so dass eine chirurgische Revaskularisation auch in Fällen ohne Anzeichen einer Myokardischämie empfohlen wird."},"es":{"name":"Origen anómalo de la arteria coronaria izquierda","synonyms":["AOLCA","Arteria coronaria izquierda en el seno aórtico derecho","L-ACAOS"],"definition":"Es una malformación congénita poco frecuente de las arterias coronarias caracterizada por un origen y un curso anómalo de la arteria coronaria izquierda, que se origina en el seno de Valsalva derecho 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 riesgo elevado de muerte súbita cardíaca, por lo que se suele recomendar la revascularización quirúrgica, incluso en los casos en los que no hay evidencias asociadas de isquemia miocárdica. Evidementemente, la decisión debe ser individualizada."},"it":{"name":"Origine anomala dell'arteria coronaria sinistra dall'aorta","definition":"Si tratta di una malformazione congenita rara dell'arteria coronaria, caratterizzata da anomalie dell'origine e del decorso dell'arteria coronaria sinistra, che origina dal seno aortico di Valsalva destro 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 elevato di morte cardiaca improvvisa; perciò, si raccomanda la rivascolarizzazione chirurgica, anche in assenza di una evidente ischemia del miocardio."},"nl":{"name":"Aberrante oorsprong van linkerkransslagader in aorta","synonyms":["AOLCA","L-ACAOS","Linkerkransslagader uit rechter sinus van Valsalva"],"definition":"Een zeldzame, congenitale malformatie van de kransslagaders, gekarakteriseerd door een aberrante oorsprong en een aberrant verloop van de linkerkransslagader, die ontspringt uit de rechter 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 hoog risico op plotse hartdood, dus chirurgische revascularisatie wordt aanbevolen, zelfs in gevallen zonder geassocieerd bewijs van myocardiale ischemie."},"pt":{"name":"Artéria coronária esquerda com origem aórtica anómala"},"pl":{"name":"Nieprawidłowe odejście lewej tętnicy wieńcowej od aorty","synonyms":["AOLCA","L-ACAOS","Left coronary artery from right aortic sinus"]},"cs":{"name":"Anomální aortální odstup levé koronární tepny","synonyms":["AOLCA","L-ACAOS","Levá koronární tepna z pravého aortálního sinu"]},"uk":{"name":"Аномальне відходження лівої коронарної артерії від аорти","synonyms":["AOLCA","L-ACAOS","Ліва коронарна артерія від правого аортального синуса"],"definition":"A rare coronary artery congenital malformation characterized by an anomalous origin and course of the left coronary artery, which originates from the right 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 high risk of sudden cardiac death so surgical revascularization is recommended even in cases with no associated evidence of myocardial ischemia."},"zh":{"name":"主动脉起源性左冠状动脉起源异常","synonyms":["左冠状动脉起源于右主动脉窦"],"definition":"一种罕见的冠状动脉先天性畸形，其特征是左冠状动脉的起源和走向异常，左冠状动脉起源于Valsalva右主动脉窦，近端走向异常，可为腔内、腔前、肺下、主动脉后、心后或包绕心尖部。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）。这种畸形与高风险心脏性猝死有关，因此即使没有心肌缺血的相关证据，也建议进行外科血管重建手术。"},"tr":{"name":"Sol koroner arterin anormal aort orijini","synonyms":["AOLCA","L-ACAOS","Sağ aort sinüsünden sol 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":"350108987"},{"source":"MONDO","code":"0035249","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3532026","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}