{"orpha":3427,"name":"Double outlet left ventricle","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["DOLV"],"definition":"Double-outlet left ventricle (DOLV) is an extremely rare congenital cardiac malformation in which both the aorta and the pulmonary artery arise, either exclusively or predominantly, from the morphologic left ventricle.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-3427/","orphanet_url":"https://www.orpha.net/en/disease/detail/3427","names":{"fr":{"name":"Ventricule gauche à double issue","synonyms":["VGDI"],"definition":"Le ventricule gauche à double issue (VGDI) est une malformation cardiaque congénitale extrêmement rare dans laquelle l'aorte et l'artère pulmonaire prennent leur origine, exclusivement ou en grande partie, dans le ventricule gauche morphologique."},"de":{"name":"Doppelausstromventrikel, linker","synonyms":["DOLV"]},"es":{"name":"Doble salida ventricular izquierda","synonyms":["DOLV"],"definition":"La doble salida ventricular izquierda (DOLV) es una malformación cardíaca extremadamente rara en la cual tanto la aorta como la arteria pulmonar surgen, ya sea en su totalidad o predominantemente, desde el ventrículo morfológicamente izquierdo."},"it":{"name":"Ventricolo sinistro a doppia uscita","synonyms":["DOLV"],"definition":"Il ventricolo sinistro a doppia uscita (DOLV) è una malformazione cardiaca congenita estremamente rara, in cui sia l'aorta che l'arteria polmonare originano, in modo esclusivo o predominante, dal ventricolo sinistro morfologico."},"nl":{"name":"Linkerventrikel met dubbele uitstroom","synonyms":["DOLV","Double outlet linkerventrikel"],"definition":"Dubbele-uitstroom linkerventrikel (DOLV) is een zeer zeldzame aangeboren hartafwijking, waarbij zowel de aorta als de longslagader, volledig of grotendeels, ontspringen uit de morfologische linkerhartkamer."},"pt":{"name":"Ventrículo esquerdo com dupla saída"},"pl":{"name":"Dwuujściowa lewa komora","synonyms":["DOLV"]},"cs":{"name":"Dvojvýtoková levá komora","synonyms":["DOLV"]},"uk":{"name":"Подвійне відходження великих артерій від лівого шлуночка","synonyms":["DOLV"],"definition":"Double-outlet left ventricle (DOLV) is an extremely rare congenital cardiac malformation in which both the aorta and the pulmonary artery arise, either exclusively or predominantly, from the morphologic left ventricle."},"zh":{"name":"左室双出口","synonyms":["DOLV"]},"tr":{"name":"Çift çıkışlı sol ventrikül","synonyms":["DOLV"]}},"xrefs":[{"source":"ICD-10","code":"Q20.2","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"LA85.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2094997989"},{"source":"MONDO","code":"0018090","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0265809","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1907","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10080133","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":2445,"name":"Conotruncal heart malformations","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Worldwide","status":"Validated","source":"21106011[PMID]_22644696[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2023-02-01","source":"21106011[PMID]_31468015[PMID]_36451474[PMID]_25050529[PMID]","terms":[{"hpo":"HP:0011581","label":"Double outlet left ventricle","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0033118","label":"Abnormal right ventricular function","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001643","label":"Patent ductus arteriosus","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001629","label":"Ventricular septal defect","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011686","label":"Abnormal coronary artery course","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001640","label":"Cardiomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000961","label":"Cyanosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011662","label":"Tricuspid atresia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000028","label":"Cryptorchidism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000316","label":"Hypertelorism","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000202","label":"Orofacial cleft","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031664","label":"Systolic heart murmur","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002789","label":"Tachypnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005182","label":"Bicuspid pulmonary valve","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0004415","label":"Pulmonary artery stenosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0010882","label":"Pulmonary valve atresia","frequency":"Very rare (<4-1%)","criteria":null}]}}