{"orpha":99099,"name":"Cor triatriatum sinister","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Cor triatriatum sinistrum","Divided left atrium"],"definition":"A rare, congenital, non-syndromic, heart malformation characterized by the presence of a thin, fibromuscular membrane subdividing the left atrium into an upper and lower chamber. The upper chamber receives blood from the pulmonary veins and the lower chamber is attached to the left atrial appendage. Therefore, the membrane blocks the orifice of the mitral valve and leads to obstruction of the left ventricular inflow. It may be asymptomatic or present in infancy with tachypnea, dyspnea, hemoptysis, chest pain, syncope, pulmonary edema, pulmonary hypertension, or heart failure, depending on the degree of obstruction. The anomaly may be isolated or associated with other congenital heart anomalies.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99099/","orphanet_url":"https://www.orpha.net/en/disease/detail/99099","names":{"fr":{"name":"Coeur triatrial gauche","synonyms":["Cor triatriatum sinister","Division de l'atrium gauche","Division de l'oreillette gauche"],"definition":"Malformation cardiaque congénitale non syndromique rare caractérisée par la présence d'une fine membrane fibromusculaire subdivisant l'oreillette gauche en une chambre supérieure et une chambre inférieure. La chambre supérieure reçoit le sang en provenance des veines pulmonaires et la chambre inférieure est rattachée à l'appendice auriculaire gauche. Par conséquent, la membrane bloque l'orifice mitral et fait obstacle au flux entrant du ventricule gauche. Elle peut être asymptomatique ou se manifester dans la petite enfance par une tachypnée, une dyspnée, une hémoptysie, des douleurs thoraciques, une syncope, un oedème pulmonaire, une hypertension pulmonaire ou une insuffisance cardiaque, selon le degré d'obstruction. L'anomalie peut être isolée ou associée à d'autres malformations cardiaques congénitales."},"de":{"name":"Cor triatriatum sinister","synonyms":["Geteilter linker Vorhof"],"definition":"Eine angeborene nicht-syndromale Herzfehlbildung, die durch das Vorhandensein einer dünnen fibromuskulären Membran gekennzeichnet ist, die den linken Vorhof in eine obere und eine untere Kammer unterteilt. Die obere Kammer wird von den Lungenvenen mit Blut versorgt, während die untere Kammer mit dem linken Vorhofanhang verbunden ist. Daher blockiert die Membran die Öffnung der Mitralklappe und führt zu einer Obstruktion des linksventrikulären Zuflusses. Je nach Grad der Obstruktion kann die Anomalie asymptomatisch sein oder im Säuglingsalter mit Tachypnoe, Dyspnoe, Bluthusten, Brustschmerzen, Synkope, Lungenödem, pulmonaler Hypertonie oder Herzversagen auftreten. Die Anomalie kann isoliert oder in Verbindung mit anderen kongenitalen Herzanomalien auftreten."},"es":{"name":"Cor triatriatum sinister","synonyms":["Aurícula izquierda dividida","Cor triatriatum sinistrum"],"definition":"Es una malformación cardíaca no sindrómica, congénita y poco frecuente, caracterizada por la presencia de una membrana fibromuscular delgada que subdivide la aurícula izquierda en una cámara superior e inferior. La cámara superior recibe sangre de las venas pulmonares y la inferior está conectada a la orejuela auricular izquierda. De esta manera, la membrana bloquea el orificio de la válvula mitral, resultando en la obstrucción del tracto de entrada del ventrículo izquierdo. Según el grado de obstrucción puede ser asintomático o presentarse en lactantes con taquipnea, disnea, hemoptisis, dolor torácico, síncope, edema pulmonar, hipertensión pulmonar o insuficiencia cardíaca. La anomalía puede presentarse aislada o asociada a otras anomalías cardíacas congénitas."},"it":{"name":"Cor triatriatum sinister","synonyms":["Atrio sinistro diviso","Cor triatriatum sinistrum"],"definition":"Si tratta di una cardiopatia congenita rara non sindromica, caratterizzata dalla presenza di una sottile membrana fibromuscolare che suddivide l'atrio sinistro in una camera superiore e una inferiore. La camera superiore riceve il sangue dalle vene polmonari, mentre quella inferiore è attaccata all'orecchietta atriale sinistra, bloccando l'orifizio della valvola mitrale con conseguente ostruzione del flusso in ingresso nel ventricolo sinistro. I pazienti possono essere asintomatici, oppure presentare durante l'infanzia tachipnea, dispnea, emottisi, dolore toracico, sincope, edema polmonare, ipertensione polmonare o insufficienza respiratoria, a seconda del grado di ostruzione. L'anomalia può essere isolata o associata ad altre cardiopatie congenite."},"nl":{"name":"Cor triatriatum sinister","synonyms":["Cor triatriatum sinistrum","Verdeeld linkeratrium"],"definition":"Een zeldzame, congenitale, niet-syndromale hartmalformatie gekarakteriseerd door de aanwezigheid van een dun, fibromusculair membraan dat het linkeratrium verdeeld in een bovenste en onderste kamer. De bovenste kamer ontvangt bloed van de longaders, en de onderste kamer is vastgehecht aan het linkerhartoor. Het membraan blokkeert dus de opening van de mitralisklep en leidt tot obstructie van de instroom in het linkerventrikel. Het kan asymptomatisch of zich presenteren in de zuigelingentijd met tachypneu, dyspneu, hemoptoë, pijn in de borst, syncope, longoedeem, pulmonale hypertensie, of hartfalen, afhankelijk van de mate van obstructie. De anomalie kan geïsoleerd voorkomen of geassocieerd zijn met andere congenitale hartanomalieën."},"pt":{"name":"Cor triatriatum"},"pl":{"name":"Serce trójprzedsionkowe lewe","synonyms":["Podzielony lewy przedsionek"]},"cs":{"name":"Cor triatriatum sinister","synonyms":["Cor triatriatum sinistrum","Rozdělená levá síň"]},"uk":{"name":"Трьохпередсердне серце (ліве)","synonyms":["Cor triatriatum sinistrum","Розділене ліве передсердя"],"definition":"A rare, congenital, non-syndromic, heart malformation characterized by the presence of a thin, fibromuscular membrane subdividing the left atrium into an upper and lower chamber. The upper chamber receives blood from the pulmonary veins and the lower chamber is attached to the left atrial appendage. Therefore, the membrane blocks the orifice of the mitral valve and leads to obstruction of the left ventricular inflow. It may be asymptomatic or present in infancy with tachypnea, dyspnea, hemoptysis, chest pain, syncope, pulmonary edema, pulmonary hypertension, or heart failure, depending on the degree of obstruction. The anomaly may be isolated or associated with other congenital heart anomalies."},"zh":{"name":"左侧三房心","synonyms":["左心房三间隔"],"definition":"该病是一种罕见的、先天性的、非综合征性的心脏畸形，其特征是存在一层薄薄的纤维肌膜，将左心房分成上下两个腔。上腔接受来自肺静脉的血液，下腔与左心耳相连。此膜堵塞了二尖瓣的开口，导致左心室流入道阻塞。根据梗阻程度的不同，可表现为无症状或在婴儿期出现呼吸急促、呼吸困难、咯血、胸痛、晕厥、肺水肿、肺动脉高压或心力衰竭。这种异常可能是孤立的，也可能与其他先天性心脏畸形相关。"},"tr":{"name":"Kor triatriatum sinister","synonyms":["Bölünmüş sol atriyum","Kor triatriatum sinistrum"]}},"xrefs":[{"source":"ICD-10","code":"Q24.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA8G.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"90967508"},{"source":"MONDO","code":"0020430","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0344712","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12484","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":1463,"name":"Triatrial heart","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Childhood","Infancy"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}