{"orpha":99117,"name":"Coronary sinus stenosis","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare congenital anomaly of the coronary sinus characterized by its stenosis at the ostium, lumen, or origin, typically leading to dilation of the vessel. Symptoms are variable and can include palpitations, tachypnea, dyspnea, chest pain, fatigue, and cyanosis. The malformation may be associated with other cardiac anomalies, such as coronary artery-coronary sinus fistula, unroofed coronary sinus, atrial septal defect, coronary sinus-left atrium fistula, total anomalous pulmonary venous connection, and ventricular septal defect.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99117/","orphanet_url":"https://www.orpha.net/en/disease/detail/99117","names":{"fr":{"name":"Sténose du sinus coronaire","definition":"Anomalie congénitale rare du sinus coronaire caractérisée par sa sténose au niveau de l'ostium, du lumen ou de sa naissance, entraînant généralement une dilatation du vaisseau. Les symptômes sont variables et peuvent inclure des palpitations, une tachypnée, une dyspnée, des douleurs thoraciques, une fatigue et une cyanose. La maladie peut être associée à d'autres malformations cardiaques, telles qu'une fistule entre l'artère coronaire et le sinus coronaire, un sinus coronaire non recouvert, une communication interauriculaire, une fistule entre le sinus coronaire et l'oreillette gauche, une communication veineuse pulmonaire anormale totale et une communication interventriculaire."},"de":{"name":"Koronarsinusstenose","definition":"Eine seltene angeborene Anomalie des Koronarsinus, die durch eine Verengung am Ostium, Lumen oder Ursprung gekennzeichnet ist und typischerweise zu einer Dilatation des Gefäßes führt. Die Symptome sind unterschiedlich und können Herzklopfen, Tachypnoe, Dyspnoe, Brustschmerzen, Müdigkeit und Zyanose umfassen. Die Fehlbildung kann mit anderen Herzfehlern einhergehen, z. B. mit einer Koronarsinusfistel, einem nicht abgedeckten Koronarsinus, einem Vorhofseptumdefekt, einer linksatrialen Koronarsinusfistel, einer völlig abnormalen Pulmonalvenenverbindung und einem Ventrikelseptumdefekt."},"es":{"name":"Estenosis del seno coronario","definition":"Es una anomalía congénita poco frecuente del seno coronario caracterizada por estenosis del ostium, la luz o el origen, que conduce habitualmente a la dilatación del vaso. Los síntomas son variables y pueden incluir palpitaciones, taquipnea, disnea, dolor torácico, cansancio y cianosis. La malformación puede asociarse con otras anomalías cardíacas, como fístula arteria coronaria-seno coronario, seno coronario ''sin techo '', comunicación interauricular, fístula seno coronario-aurícula izquierda, drenaje venoso pulmonar anómalo total y comunicación interventricular."},"it":{"name":"Stenosi del seno coronarico","definition":"Si tratta di un'anomalia congenita rara del seno coronarico, caratterizzata da una stenosi a livello dell'ostio, del lume o del punto di origine, che di solito causa la dilatazione del vaso. I sintomi sono variabili e comprendono le palpitazioni, la tachipnea, la dispnea, il dolore toracico, l'affaticamento e la cianosi. La malformazione può associarsi ad altre cardiopatie, come la fistola arteria coronaria-seno coronarico, il seno coronarico scoperto, il difetto del setto interatriale, la fistola seno coronarico-atrio sinistro, la connessione venosa polmonare anomala totale e il difetto del setto interventricolare."},"nl":{"name":"Coronaire sinusstenose","definition":"Een zeldzame congenitale anomalie van sinus coronarius, gekarakteriseerd door stenose ervan ter hoogte van ostium, lumen, of oorsprong, doorgaans met dilatatie van het bloedvat tot gevolg. Symptomen zijn variabel, met onder meer hartkloppingen, tachypneu, dyspneu, pijn op borst, vermoeidheid, en cyanose. De malformatie kan geassocieerd zijn met andere hartanomalieën, zoals fistel tussen kransslagader en sinus coronarius, afwezigheid van sinus coronarius, atriumseptumdefect, fistel tussen sinus coronarius en linkeratrium, totale afwijkende pulmonale veneuze verbinding, en ventrikelseptumdefect."},"pt":{"name":"Estenose do seio coronário"},"pl":{"name":"Zwężenie zatoki wieńcowej"},"cs":{"name":"Stenóza koronárního sinu"},"uk":{"name":"Стеноз коронарного синуса","definition":"A rare congenital anomaly of the coronary sinus characterized by its stenosis at the ostium, lumen, or origin, typically leading to dilation of the vessel. Symptoms are variable and can include palpitations, tachypnea, dyspnea, chest pain, fatigue, and cyanosis. The malformation may be associated with other cardiac anomalies, such as coronary artery-coronary sinus fistula, unroofed coronary sinus, atrial septal defect, coronary sinus-left atrium fistula, total anomalous pulmonary venous connection, and ventricular septal defect."},"zh":{"name":"冠状静脉窦狭窄"},"tr":{"name":"Koroner sinüs darlığı"}},"xrefs":[{"source":"ICD-10","code":"Q21.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA86.Y","qualifier":"BTNT","icd_relation":"index","validated":true,"icd11_uri":"1460525276"},{"source":"MONDO","code":"0020446","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0344684","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":95500,"name":"Congenital anomaly of the coronary sinus","classifications":[147,148,198]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}