{"orpha":99098,"name":"Cor triatriatum dexter","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Cor triatriatum dextrum","Divided right atrium"],"definition":"A rare, congenital, non-syndromic, heart malformation characterized by the persistence of the embryonic right valve of the sinus venosus which results in a subdivision of right atrium into two chambers. Clinical manifestations depend on the degree of right atrial septation and the size of sinoatrial orifice and vary from asymptomatic to symptoms of tricuspid valve stenosis, atrial fibrillation, cyanosis, syncope, elevated central venous pressure and right heart failure. The anomaly may be isolated or associated with other congenital heart anomalies.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99098/","orphanet_url":"https://www.orpha.net/en/disease/detail/99098","names":{"fr":{"name":"Coeur triatrial droit","synonyms":["Cor triatriatum dexter","Cor triatriatum dextrum","Division de l'atrium droit","Division de l'oreillette droite"],"definition":"Malformation cardiaque congénitale non syndromique rare caractérisée par la persistance de la valvule droite du sinus veineux, un vestige embryonnaire entraînant une subdivision de l'oreillette droite en deux chambres. Le tableau clinique dépend du degré de septation auriculaire droite et de la taille de l'orifice sino-auriculaire et oscille entre la forme asymptomatique et les formes symptomatiques à type de sténose de la valve tricuspide, de fibrillation auriculaire, de cyanose, de syncope, de pression veineuse centrale élevée et d'insuffisance cardiaque droite. L'anomalie peut être isolée ou associée à d'autres malformations cardiaques congénitales."},"de":{"name":"Cor triatriatum dexter","synonyms":["Geteilter rechter Vorhof"],"definition":"Eine seltene, angeborene, nicht-syndromale Herzfehlbildung, die durch die Persistenz der embryonalen rechten Klappe des Sinus venosus gekennzeichnet ist und zu einer Unterteilung des rechten Vorhofs in zwei Kammern führt. Die klinischen Manifestationen hängen vom Grad der rechten Vorhofseptation und der Größe der sinoatrialen Öffnung ab und variieren von asymptomatisch bis hin zu Symptomen der Trikuspidalklappenstenose, Vorhofflimmern, Zyanose, Synkope, erhöhtem zentralem Venendruck und Rechtsherzversagen. Die Anomalie kann isoliert oder mit anderen kongenitalen Herzanomalien assoziiert sein."},"es":{"name":"Cor triatriatum dexter","synonyms":["Aurícula derecha dividida","Cor triatriatum dextrum"],"definition":"Es una malformación cardíaca no sindrómica, congénita y poco frecuente, caracterizada por la persistencia de la válvula derecha embrionaria del seno venoso, resultando en la subdivisión de la aurícula derecha en dos cámaras. Las manifestaciones clínicas dependen del grado de septación auricular derecha y del tamaño del orificio sinoauricular, variando desde asintomática a síntomas secundarios a estenosis de la válvula tricúspide, fibrilación auricular, cianosis, síncope, presión venosa central elevada e insuficiencia cardíaca derecha. Puede presentarse de forma aislada o asociada a otras anomalías cardíacas congénitas."},"it":{"name":"Cor triatriatum dexter","synonyms":["Atrio destro diviso","Cor triatriatum dextrum"],"definition":"Si tratta di una cardiopatia congenita rara non sindromica, caratterizzata dalla persistenza della valvola embrionale destra del seno venoso, che esita nella suddivisione dell'atrio destro in due camere. I sintomi clinici dipendono dal grado di settazione dell'atrio destro e dalle dimensioni dell'orifizio seno-atriale. I pazienti possono essere asintomatici oppure presentare i sintomi della stenosi della valvola tricuspide, fibrillazione atriale, cianosi, sincope, pressione venosa centrale elevata e insufficienza della parte destra del cuore. L'anomalia può essere isolata o associata ad altre cardiopatie congenite."},"nl":{"name":"Cor triatriatum dexter","synonyms":["Cor triatriatum dextrum","Verdeeld rechteratrium"],"definition":"Een zeldzame, congenitale, niet-syndromale hartmalformatie gekarakteriseerd door persistentie van de embryonale rechterklep van de sinus venosus, wat leidt tot een onderverdeling van het rechteratrium in twee kamers. Klinische manifestaties zijn afhankelijk van de mate van septatie van het rechteratrium en de omvang van de sinoatriale opening, en variëren van asymptomatisch tot symptomen tricuspidalisklepstenose, atriumfibrillatie, cyanose, syncope, verhoogde centrale veneuze druk en rechter hartfalen. De anomalie kan geïsoleerd voorkomen of geassocieerd zijn met andere congenitale hartanomalieën."},"pt":{"name":"Cor triatriatum destro"},"pl":{"name":"Serce trójprzedsionkowe prawe","synonyms":["Prawy przedsionek podzielony"]},"cs":{"name":"Cor triatriatum dexter","synonyms":["Cor triatriatum dextrum","Rozdělená pravá síň"]},"uk":{"name":"Трьохпередсердне серце (праве)","synonyms":["Cor triatriatum dextrum","Розділене праве передсердя"],"definition":"A rare, congenital, non-syndromic, heart malformation characterized by the persistence of the embryonic right valve of the sinus venosus which results in a subdivision of right atrium into two chambers. Clinical manifestations depend on the degree of right atrial septation and the size of sinoatrial orifice and vary from asymptomatic to symptoms of tricuspid valve stenosis, atrial fibrillation, cyanosis, syncope, elevated central venous pressure and right heart failure. The anomaly may be isolated or associated with other congenital heart anomalies."},"zh":{"name":"右侧三房心","synonyms":["右心房三间隔"],"definition":"该病是一种罕见的、先天性的、非综合征的心脏畸形，其特征是静脉窦的胚胎右瓣膜持续存在，导致右心房分成两个房间。临床表现取决于右心房间隔的程度和窦房口的大小，从无症状到三尖瓣狭窄、心房颤动、紫绀、晕厥、中心静脉压升高和右心衰竭的症状不等。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 dekster","synonyms":["Bölünmüş sağ atriyum","Kor triatriatum dekstrum"]}},"xrefs":[{"source":"ICD-10","code":"Q24.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA8F","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"294192583"},{"source":"MONDO","code":"0020429","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0344697","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12483","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}