{"orpha":99119,"name":"Right inferior vena cava connecting to left-sided atrium","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Right IVC connecting to left-sided atrium","Right inferior caval vein connecting to left-sided atrium"],"definition":"A rare vascular anomaly characterized by a congenital anomalous connection between the inferior vena cava and the left atrium. Clinical manifestations depend on the presence and nature of additional cardiac defects and include cyanosis, dyspnea, failure to thrive, clubbing of fingers and toes, and potentially heart failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99119/","orphanet_url":"https://www.orpha.net/en/disease/detail/99119","names":{"fr":{"name":"Drainage anormal de la veine cave inférieure dans l'oreillette gauche","definition":"Anomalie vasculaire rare, caractérisée par une connexion congénitale anormale entre la veine cave inférieure et l'oreillette gauche. Les manifestations cliniques dépendent du type des autres anomalies cardiaques présentes et peuvent inclure cyanose, dyspnée, retard de croissance, hippocratisme des doigts et des orteils et éventuellement insuffisance cardiaque."},"de":{"name":"Rechte Vena cava inferior mit Mündung in den linken Vorhof","synonyms":["Rechte untere Hohlvene mit Mündung in den linken Vorhof"],"definition":"Eine seltene Gefäßanomalie, die durch eine angeborene anomale Verbindung zwischen der Vena cava inferior und dem linken Vorhof gekennzeichnet ist. Die klinischen Manifestationen hängen vom Vorhandensein und der Art zusätzlicher kardialer Defekte ab und umfassen Zyanose, Dyspnoe, Gedeihstörung, Keulenbildung an Fingern und Zehen und möglicherweise Herzversagen."},"es":{"name":"Vena cava inferior derecha conectada a la aurícula izquierda","synonyms":["VCI derecha conectada a la aurícula izquierda"],"definition":"Es una anomalía vascular poco frecuente caracterizada por una conexión anómala congénita entre la vena cava inferior y la aurícula izquierda. Las manifestaciones clínicas dependen de la presencia y de la naturaleza de los defectos adicionales, incluyendo cianosis, disnea, fallo de medro, hipocratismo de los dedos de las manos y de los pies y, potencialmente, insuficiencia cardíaca."},"it":{"name":"Vena cava inferiore destra collegata all'atrio sinistro","definition":"Si tratta di un'anomalia vascolare rara, caratterizzata da una connessione anomala congenita tra la vena cava inferiore e l'atrio sinistro. I segni clinici dipendono dall'eventuale presenza e dalla natura di altre cardiopatie associate, e comprendono la cianosi, la dispnea, il ritardo della crescita, l'ippocratismo delle dita delle mani e dei piedi, e l'insufficienza cardiaca."},"nl":{"name":"Rechter vena cava inferior verbonden met linkeratrium","synonyms":["Rechter VCI verbonden met linkeratrium","Rechter onderste holle ader verbonden met linkeratrium"],"definition":"Een zeldzame vasculaire anomalie, gekenmerkt door een congenitale abnormale verbinding tussen vena cava inferior en linkeratrium. Klinische manifestaties zijn afhankelijk van de aanwezigheid en de aard van bijkomende hartdefecten, en bestaan onder meer uit cyanose, dyspneu, niet-gedijen, trommelstokvingers en -tenen, en mogelijk hartfalen."},"pt":{"name":"VCI, direita, ligando ao lado esquerdo auricular"},"pl":{"name":"Prawa żyła główna dolna uchodząca do lewego przedsionka","synonyms":["Prawa IVC uchodząca do lewego przedsionka","Prawa żyła główna dolna łącząca się z lewostronnym przedsionkiem"]},"cs":{"name":"Pravá dolní dutá žíla ústící do levé síně","synonyms":["Pravá VCI napojující se na levostrannou síň","Pravá dolní dutá žíla napojujicí se na levostrannou síň"]},"uk":{"name":"Права нижня порожниста вена, яка дренується в ліворозташоване передсердя","synonyms":["Права нижня порожниста вена зєднується з лівим передсердям","Права вентрикулярна вена зєднується з лівим передсердям"],"definition":"A rare vascular anomaly characterized by a congenital anomalous connection between the inferior vena cava and the left atrium. Clinical manifestations depend on the presence and nature of additional cardiac defects and include cyanosis, dyspnea, failure to thrive, clubbing of fingers and toes, and potentially heart failure."},"zh":{"name":"右下腔静脉连接左心房","synonyms":["右侧下腔静脉连接左心房"]},"tr":{"name":"Sol taraftaki atriyuma bağlanan sağ inferior vena kava","synonyms":["Sol taraftaki atriyuma bağlanan sağ IVC","Sol taraftaki atriyuma bağlanan sağ inferior kaval ven"]}},"xrefs":[{"source":"ICD-10","code":"Q26.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA86.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0020448","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3164330","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":95499,"name":"Congenital anomaly of the inferior vena cava","classifications":[147,148,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}