{"orpha":99064,"name":"Straddling and/or overriding mitral valve","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, congenital, non-syndromic heart malformation characterized by an abnormal attachment of the mitral chordae to both ventricles. Straddling mitral valve is usually associated with conotruncal anomalies, most commonly double outlet right ventricle or transposition of the great arteries. Overriding mitral valve is characterized by a mitral annulus committed to the two ventricular chambers, where the mitral valve is shared between the ventricles. Straddling and overriding mitral valve can occur together or in isolation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99064/","orphanet_url":"https://www.orpha.net/en/disease/detail/99064","names":{"fr":{"name":"Straddling et/ou overriding de la valve mitrale","definition":"Malformation cardiaque congénitale non syndromique rare caractérisée par une anomalie de fixation des cordages tendineux de la valve mitrale aux deux ventricules. La valve mitrale chevauchante (dite <i>straddling</i>) est généralement associée à des malformations conotroncales, dont les plus fréquentes sont un ventricule droit à double sortie ou une transposition des gros vaisseaux. La valve mitrale en surplomb (<i>overriding</i>) est caractérisée par un anneau mitral qui s'abouche dans les deux chambres ventriculaires, la valve mitrale étant ainsi répartie entre les ventricules. Les <i>straddling</i> et <i>overriding</i> de la valve mitrale peuvent être associés ou isolés."},"de":{"name":"Mitralklappe, gespreizte und/oder reitende","definition":"Eine seltene, angeborene, nicht-syndromale Fehlbildung des Herzens, die durch eine abnorme Befestigung der Mitralchordae an beiden Ventrikeln gekennzeichnet ist. Die Straddling-Mitralklappe ist in der Regel mit konotrunkalen Anomalien assoziiert, am häufigsten mit einem doppelten rechten Ventrikel oder einer Transposition der großen Arterien. Die überschiebende Mitralklappe ist durch einen Mitralanulus gekennzeichnet, der an die beiden Ventrikelkammern gebunden ist, wobei die Mitralklappe von den Ventrikeln gemeinsam genutzt wird. Straddling und Overriding der Mitralklappe können gemeinsam oder isoliert auftreten."},"es":{"name":"Cabalgamiento de la válvula mitral","definition":"Es una malformación cardíaca congénita, no sindrómica y poco frecuente, caracterizada por una fijación anómala de las cuerdas mitrales a ambos ventrículos. Por lo general, el ''straddling'' mitral se asocia con anomalías conotruncales, incluyendo más frecuentemente la doble salida del ventrículo derecho o la transposición de las grandes arterias. El cabalgamiento de la válvula mitral está caracterizado por un anillo mitral comprometido con ambas cámaras ventriculares con una única válvula mitral compartida entre los dos ventrículos. Ambos tipos de malformaciones en la válvula mitral pueden ocurrir simultáneamente o de forma aislada."},"it":{"name":"Valvola mitrale \"a cavallo\" e/o \"con sconfinamento\"","definition":"È una cardiopatia congenita rara non sindromica, caratterizzata da un'attaccatura anomala delle corde mitraliche ad entrambi i ventricoli. La valvola mitrale ''a cavallo'' di solito si associa ad anomalie troncoconali, in particolare il ventricolo destro a doppia uscita o la trasposizione delle grandi arterie. La valvola mitrale ''con sconfinamento'' è caratterizzata dalla presenza di un anello mitralico connesso a entrambe le camere ventricolari, con la valvola mitrale condivisa dai ventricoli. Le due anomalie possono essere isolate o concomitanti."},"nl":{"name":"Gespreide en/of overrijdende mitralisklep","definition":"Een zeldzame, congenitale, niet-syndromale hartmalformatie gekarakteriseerd door abnormale aanhechting van de chordae tendineae (peesdraden) van de mitralisklep aan beide ventrikels. Gespreide mitralisklep is meestal geassocieerd met conotruncale anomalieën, doorgaans dubbele uitstroom uit het rechterventrikel of transpositie van de grote slagaders. Overrijdende mitralisklep wordt gekarakteriseerd door een mitralisannulus verbonden met de twee hartkamers, waarbij de mitralisklep wordt gedeeld door beide ventrikels. Gespreide en overrijdende mitralisklep kunnen samen of geïsoleerd voorkomen."},"pt":{"name":"Válvula mitral, straddling e/ou overriding"},"pl":{"name":"Anomalia zastawki dwudzielnej typu straddling lub(i) overriding"},"cs":{"name":"Široká a/nebo nasedající mitrální chlopeň"},"uk":{"name":"Straddling and/or overriding мітрального клапана (аномаіля розташування хорд та площини атріовентрикулярних клапанів по відношенню до відповідних шлуночків)","definition":"A rare, congenital, non-syndromic heart malformation characterized by an abnormal attachment of the mitral chordae to both ventricles. Straddling mitral valve is usually associated with conotruncal anomalies, most commonly double outlet right ventricle or transposition of the great arteries. Overriding mitral valve is characterized by a mitral annulus committed to the two ventricular chambers, where the mitral valve is shared between the ventricles. Straddling and overriding mitral valve can occur together or in isolation."},"zh":{"name":"二尖瓣骑跨/跨位","definition":"该病是一种罕见的、先天性的、无综合征的心脏畸形，其特征是二尖瓣腱索与两个心室不正常连接。骑跨二尖瓣通常伴有圆锥干畸形，最常见的是右心室双出口或大动脉转位。二尖瓣重叠的特征是二尖瓣环连接到两个心室，在那里二尖瓣在两个心室之间共享。骑跨二尖瓣和二尖瓣重叠可以同时发生，也可以单独发生。"},"tr":{"name":"Straddling ve/veya overriding mitral kapak"}},"xrefs":[{"source":"ICD-10","code":"Q23.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA87.1Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"462079876"},{"source":"MONDO","code":"0020405","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681625","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":95465,"name":"Cleft mitral valve","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}