{"orpha":99107,"name":"Atrial septal aneurysm","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare congenital non-syndromic heart malformation characterized by an abnormal protrusion of the interatrial septum into the right or left atrium, or both, during the cardiorespiratory cycle. The defect may be limited to the fossa ovalis or involve the entire septum. It can present as an isolated finding but is more often associated with interatrial shunts, in particular patent foramen ovale. Clinically it increases the risk of peripheral arterial embolism and stroke.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99107/","orphanet_url":"https://www.orpha.net/en/disease/detail/99107","names":{"fr":{"name":"Anévrisme du septum interauriculaire","definition":"Malformation cardiaque congénitale non syndromique rare caractérisée par une protrusion anormale du septum interatrial dans l'oreillette droite et/ou gauche, lors du cycle cardio-respiratoire. La malformation peut être limitée au foramen ovale ou concerner l'ensemble du septum. Associée le plus souvent à un shunt droit-gauche, en particulier en cas d'un foramen ovale perméable, la maladie peut se présenter également sous une forme isolée. Sur le plan clinique, la malformation augmente le risque d'embolie artérielle périphérique et d'accident vasculaire cérébral."},"de":{"name":"Atrialseptumaneurysma","definition":"Eine angeborene nicht-syndromale Fehlbildung des Herzens, die durch eine abnorme Vorwölbung der Vorhofscheidewand in den rechten oder linken Vorhof oder in beide Vorhöfe während des kardiorespiratorischen Zyklus gekennzeichnet ist. Der Defekt kann auf die Fossa ovalis beschränkt sein oder das gesamte Septum betreffen. Er kann als isolierter Befund auftreten, ist jedoch häufiger mit interatrialen Shunts verbunden, insbesondere mit dem offenen Foramen ovale. Klinisch gesehen erhöht er das Risiko einer peripheren arteriellen Embolie und eines Schlaganfalls."},"es":{"name":"Aneurisma del septo auricular","synonyms":["Aneurisma del septo atrial"],"definition":"Es una malformación cardíaca congénita no sindrómica poco frecuente caracterizada por un desplazamiento anómalo del tabique interauricular hacia la aurícula derecha o izquierda, o ambas, durante el ciclo cardiorrespiratorio. El defecto puede limitarse a la fosa oval o afectar a todo el tabique. Puede presentarse como un hallazgo aislado, aunque se asocia con mayor frecuencia con la presencia de cortocircuitos interauriculares, en particular con el foramen oval permeable. Clínicamente aumenta el riesgo de embolia arterial periférica y accidente cerebrovascular."},"it":{"name":"Aneurisma del setto interatriale","definition":"Si tratta di una cardiopatia congenita non sindromica rara, caratterizzata da un'anomala protrusione del setto interatriale nell'atrio destro o sinistro, o in entrambi, durante il ciclo cardiorespiratorio. Il difetto può essere limitato alla fossa ovale oppure interessare tutto il setto. Può essere isolato, ma spesso si associa a shunt interatriali, in particolare al forame ovale pervio. Dal punto di vista clinico, aumenta il rischio di embolismo arterioso periferico e di ictus."},"nl":{"name":"Aneurysma van atriumseptum","definition":"Een zeldzame congenitale niet-syndromale hartmalformatie, gekarakteriseerd door abnormale protrusie van interatriaal septum in rechter- of linkeratrium, of beide, tijdens de cardiorespiratoire cyclus. Het defect kan beperkt blijven tot fossa ovalis of het hele septum treffen. Het kan zich presenteren als een geïsoleerde bevinding, maar is vaker geassocieerd met interatriale shunts, voornamelijk persisterend foramen ovale. Klinisch is er verhoogd risico op embolie van perifere arterie en beroerte."},"pt":{"name":"Aneurisma do septo auricular"},"pl":{"name":"Tętniak przegrody międzyprzedsionkowej"},"cs":{"name":"Aneuryzma síňového septa"},"uk":{"name":"Аневризма міжпередсердної перегородки","definition":"A rare congenital non-syndromic heart malformation characterized by an abnormal protrusion of the interatrial septum into the right or left atrium, or both, during the cardiorespiratory cycle. The defect may be limited to the fossa ovalis or involve the entire septum. It can present as an isolated finding but is more often associated with interatrial shunts, in particular patent foramen ovale. Clinically it increases the risk of peripheral arterial embolism and stroke."},"zh":{"name":"房间隔动脉瘤","definition":"该病是一种罕见的先天性非综合征心脏畸形，其特征是在心动周期中，房间隔异常突入右心房或左心房或同时进入右心房或左心房，缺损可能局限于卵圆窝或累及整个鼻中隔。它可以是一个孤立的发现，但更多的是与心房间分流有关，尤其是卵圆孔未闭。在临床上，它增加了外周动脉栓塞和中风的风险。"},"tr":{"name":"Atriyal septal anevrizma"}},"xrefs":[{"source":"ICD-10","code":"Q21.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA8E.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"100700036"},{"source":"MONDO","code":"0020438","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0521533","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":98727,"name":"Rare atrial defect and interatrial communication","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}