{"orpha":541478,"name":"Anomalous aortic origin of coronary artery","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["AAOCA"],"definition":"A rare group of coronary artery congenital malformation disorders characterized by an anomalous origin and course of the left or right coronary artery, which originates from the contralateral aortic sinus of Valsalva and has an anomalous trajectory which may be: pre-pulmonary (with no hemodynamic consequences), retroaortic (with a course posterior to the aortic root and no hemodynamic consequences), interarterial (located between the aorta and the pulmonary artery and associated with a poorer prognosis), subpulmonary (with an intraconal or intraseptal course), or retrocardiac (located in the posterior atrioventricular sulcus). Clinical manifestations depend on the specific anomalous origin and course which is present, with patients being frequently asymptomatic, although nonspecific chest pain, palpitations, dizziness, dyspnea or syncope, usually following physical exertion, may be associated. Sudden death, due to compression/occlusion of the coronary artery and usually associated with, or immediately following, vigorous physical exercise, may be occasionally observed.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-541478/","orphanet_url":"https://www.orpha.net/en/disease/detail/541478","names":{"fr":{"name":"Anomalie d'origine d'une artère coronaire à partir de l'aorte","synonyms":["AAOCA"],"definition":"Groupe rare d'anomalies congénitales des artères coronaires caractérisé par une anomalie d'origine et de trajet de l'artère coronaire gauche ou droite, à partir du sinus de Valsalva controlatéral et dont le trajet, anormal, peut être prépulmonaire (sans conséquence hémodynamique), rétro-aortique (postérieur à la racine de l'aorte, sans conséquence hémodynamique), interartériel (entre l'aorte et l'artère pulmonaire, associé à un pronostic moins favorable), sous-pulmonaire (trajet intraconal ou intraseptal) ou rétrocardiaque (dans le sillon atrioventriculaire postérieur). Les manifestations cliniques dépendent de l'anomalie d'origine et de trajet en question. Les patients sont souvent asymptomatiques, bien que des douleurs thoraciques non spécifiques, des palpitations, des vertiges, une dyspnée ou une syncope, généralement après un effort, puissent être observés. Des arrêts cardiorespiratoires, dus à la compression/l'obstruction de l'artère coronaire et survenant généralement de manière concomitante à un effort intense ou peu après celui-ci, peuvent être observés dans de rares cas."},"de":{"name":"Anomaler Abgang der Koronararterie","synonyms":["AAOCA"],"definition":"Eine seltene Gruppe von angeborenen Fehlbildungen der Koronararterien, die durch einen anomalen Ursprung und Verlauf der linken oder rechten Koronararterie gekennzeichnet sind, die aus dem kontralateralen Aortensinus entspringt und eine anomale Trajektorie aufweist, die sein kann: präpulmonal (ohne hämodynamische Konsequenzen), retroaortal (mit einem Verlauf hinter der Aortenwurzel und ohne hämodynamische Konsequenzen), interarteriell (zwischen Aorta und Pulmonalarterie gelegen und mit einer schlechteren Prognose assoziiert), subpulmonal (mit einem intrakonalen oder intraseptalen Verlauf) oder retrokardial (im posterioren atrioventrikulären Sulcus gelegen). Die klinischen Manifestationen hängen vom spezifischen Fehlabgang und Verlauf ab, wobei die Patienten häufig asymptomatisch sind, obwohl unspezifische Brustschmerzen, Palpitationen, Schwindel, Dyspnoe oder Synkopen, meist nach körperlicher Anstrengung, auftreten können. Ein plötzlicher Tod, der durch Kompression/Verschluss der Koronararterie verursacht wird und in der Regel mit oder unmittelbar nach starker körperlicher Anstrengung auftritt, kann gelegentlich beobachtet werden."},"es":{"name":"Origen anómalo de las arterias coronarias","synonyms":["AAOCA"],"definition":"Es un grupo de trastornos poco frecuentes debido a una malformación congénita de las arterias coronarias caracterizados por un origen y curso anómalos de la arteria coronaria izquierda o derecha, que se origina en el seno de Valsalva contralateral y que presenta un trayecto anómalo que puede ser: prepulmonar (sin consecuencias hemodinámicas), retroaórtico (con un curso posterior a la raíz aórtica y sin consecuencias hemodinámicas), interarterial (localizada entre la aorta y la arteria pulmonar y asociada a un peor pronóstico), subpulmonar (con un curso intraconal o intraseptal), o retrocardíaco (localizada en el surco auriculoventricular posterior). Las manifestaciones clínicas dependen de la anomalía concreta, del origen y del curso, con pacientes que con frecuencia permanecen asintomáticos, y otros que pueden presentar dolor torácico inespecífico, palpitaciones, mareos, disnea o síncope, por lo general, después de un esfuerzo físico. Ocasionalmente se ha descrito muerte súbita debido a la compresión/ oclusión de la arteria coronaria, por lo general, durante el ejercicio físico vigoroso o inmediatamente después."},"it":{"name":"Origine anomala dell'arteria coronaria dall'aorta","definition":"Si tratta di un gruppo di malformazioni congenite rare dell'arteria coronaria, caratterizzate da anomalie dell'origine e del decorso dell'arteria coronaria sinistra o destra, che origina dal seno aortico di Valsalva controlaterale e ha uno sviluppo anomalo che può essere prepolmonare (senza conseguenze emodinamiche), retroaortico (con un decorso posteriore alla radice dell'aorta, senza conseguenze emodinamiche), interatriale (localizzato tra l'aorta e l'arteria polmonare; si associa a una prognosi sfavorevole), sottopolmonare (con uno sviluppo intraconale o intrasettale), o retrocardiaco (localizzato nel solco atrioventricolare posteriore). I segni clinici dipendono dalla specifica origine anomala e dal suo sviluppo; spesso i pazienti sono asintomatici, ma possono presentare anche dolore toracico aspecifico, palpitazioni, vertigini, dispnea o sincope, in genere dopo l'esercizio fisico. Occasionalmente si associa a morte improvvisa, dovuta alla compressione/occlusione dell'arteria coronaria, di solito correlata, o immediatamente successiva, a un esercizio fisico intenso."},"nl":{"name":"Aberrante oorsprong van kransslagader in aorta","synonyms":["AAOCA"],"definition":"Een zeldzame groep van congenitale malformaties van de kransslagaders gekarakteriseerd door een aberrante oorsprong en aberrant verloop van de linker- of rechterkransslagader, die ontspringt uit de contralaterale sinus van Valsalva en een aberrant traject volgt, zijnde prepulmonaal (zonder hemodynamische gevolgen), retroaortaal (met een verloop posterieur van de aortawortel en geen hemodynamische gevolgen), interarterieell (tussen de aorta en de longslagader, geassocieerd met een slechtere prognose), subpulmonaal (met een intraconaal of intraseptaal verloop), of retrocardiaal (gelokaliseerd in de posterieure of atrioventriculaire sulcus). Klinische manifestaties hangen af van de specifieke aberrante oorsprong en het aberrant verloop die aanwezig zijn, en patiënten zijn frequent asymptomatisch, hoewel niet-specifieke pijn in de borst, hartkloppingen, duizeligheid, dyspneu of syncope, meestal na fysieke inspanning, geassocieerd kan zijn. Plots overlijden, als gevolg van compressie/occlusie van de kransslagader en doorgaans geassocieerd met, of direct volgend op, forse fysieke inspanning, kan occasioneel waargenomen worden."},"pt":{"name":"Artéria coronária com origem aórtica anómala"},"pl":{"name":"Nieprawidłowe odejście tętnicy wieńcowej od aorty","synonyms":["AAOCA"]},"cs":{"name":"Anomální aortální odstup koronární tepny","synonyms":["AAOCA"]},"uk":{"name":"Аномальне відходження коронарної артерії від аорти","synonyms":["AAOCA"],"definition":"A rare group of coronary artery congenital malformation disorders characterized by an anomalous origin and course of the left or right coronary artery, which originates from the contralateral aortic sinus of Valsalva and has an anomalous trajectory which may be: pre-pulmonary (with no hemodynamic consequences), retroaortic (with a course posterior to the aortic root and no hemodynamic consequences), interarterial (located between the aorta and the pulmonary artery and associated with a poorer prognosis), subpulmonary (with an intraconal or intraseptal course), or retrocardiac (located in the posterior atrioventricular sulcus). Clinical manifestations depend on the specific anomalous origin and course which is present, with patients being frequently asymptomatic, although nonspecific chest pain, palpitations, dizziness, dyspnea or syncope, usually following physical exertion, may be associated. Sudden death, due to compression/occlusion of the coronary artery and usually associated with, or immediately following, vigorous physical exercise, may be occasionally observed."},"zh":{"name":"主动脉起源性冠状动脉起源异常","synonyms":["AAOCA"],"definition":"一组罕见的冠状动脉先天性畸形疾病，其特征是左或右冠状动脉的起源和走向异常，冠状动脉起源于对侧Valsalva主动脉窦，其走向异常可能是：前肺动脉（无血流动力学后果）、复主动脉（走向在主动脉根部后方，无血流动力学后果）、动脉间（位于主动脉和肺动脉之间，与较差的预后有关）、肺下动脉（有动脉圆锥间或室间隔内走向）或心后动脉（位于后房室沟）。临床表现取决于具体的异常起源和病程，患者常无症状，但可能伴有非特异性胸痛、心悸、头晕、呼吸困难或晕厥，通常在体力消耗后出现。由于冠状动脉受压/闭塞，通常在剧烈运动时或紧接着剧烈运动后，偶尔会出现猝死。"},"tr":{"name":"Koroner arterin anormal aort orijini","synonyms":["AAOCA"]}},"xrefs":[{"source":"UMLS","code":"C5680159","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":95493,"name":"OBSOLETE: Abnormal origin or aberrant course of coronary artery","status":"inactive"},{"type":"Referred to","orpha":99085,"name":"OBSOLETE: Coronary artery intramyocardial course","status":"inactive"},{"type":"Referred to","orpha":99086,"name":"OBSOLETE: Aortopulmonary coronary arterial course","status":"inactive"},{"type":"Referred to","orpha":99088,"name":"OBSOLETE: Intramural coronary arterial course","status":"inactive"}],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":1081,"name":"Coronary artery congenital malformation","classifications":[147,148]}],"children":[{"orpha":541443,"name":"Anomalous aortic origin of the left coronary artery","type":"Morphological anomaly"},{"orpha":541454,"name":"Anomalous aortic origin of the right coronary artery","type":"Morphological anomaly"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}