{"orpha":1461,"name":"Criss-cross heart","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":["Criss-cross atrioventricular relationships","Superoinferior ventricles","Twisted atrioventricular connections"],"definition":"Criss cross heart (CCH) is a cardiac malformation where the inflow streams of the two ventricles cross due to twisting of the heart about its major axis. The clinical features depend on the particular cardiac defects associated, like simple or corrected transposition of the great arteries and ventricular septal defects.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1461/","orphanet_url":"https://www.orpha.net/en/disease/detail/1461","names":{"fr":{"name":"Coeur croisé","synonyms":["Coeur à ventricules entrecroisés","Ventricules croisés","Ventricules supéro-inférieurs"],"definition":"Le coeur croisé est une malformation cardiaque caractérisée par le croisement des courants des deux ventricules dû à la torsion du coeur autour de son axe principal. Les manifestations cliniques dépendent des anomalies cardiaques spécifiques associées, telles qu'une transposition simple ou corrigée des gros vaisseaux et une communication interventriculaire (CIV)."},"de":{"name":"Criss-Cross-Herz","synonyms":["Atrio-ventrikuläre Verbindungen, verdrehte","Superoinferiore Ventrikellage","Überkreuzte atrioventrikuläre Verhältnisse"],"definition":"Das Criss-cross-Herz (CCH) ist eine kardiale Fehlbildung, bei der sich die Zuflüsse der beiden Ventrikel (wegen einer Verdrehung des Herzens um seine Hauptachse) überkreuzen. Die klinischen Symptome werden durch die jeweilig auftretenden Herzfehler bestimmt, z.B. einfache oder korrigierte Transposition der großen Arterien und Ventrikelseptumdefekte."},"es":{"name":"Corazón en criss-cross","synonyms":["Conexiones auriculoventriculares cruzadas","Conexiones auriculoventriculares en criss-cross","Conexiones auriculoventriculares trenzadas","Ventrículos superoinferiores"],"definition":"El corazón en criss-cross (CCH) es una malformación cardiaca en la que se entrecruzan los flujos de entrada de los dos ventrículos a causa de la torsión del corazón alrededor de su eje mayor. El cuadro clínico depende de la presencia de defectos cardiacos asociados, como la transposición simple o corregida de las grandes arterias y los defectos del tabique interventricular."},"it":{"name":"Cuore criss-cross","synonyms":["Connessioni atrioventricoalri criss-cross","Connessioni atrioventricolari alterate","Ventricoli supero-inferiori"],"definition":"Il cuore criss-cross (CCH) è una malformazione cardiaca in cui si incrociano gli afflussi dei due ventricoli a causa di una torsione del cuore attorno al suo asse maggiore. Il quadro clinico dipende dalle specifiche cardiopatie associate, come la trasposizione semplice o corretta delle grandi arterie ed i difetti del setto ventricolare."},"nl":{"name":"Criss-cross hart","synonyms":["Criss-cross atrioventriculaire connecties","Gedraaide atrioventriculaire verbindingen","Supero-inferieure ventrikels"],"definition":"Criss-cross hart (CCH) is een hartafwijking waarbij de binnenkomende bloedstromen van de twee ventrikels kruisen door een verdraaiing van het hart rond de hoofdas. De klinische kenmerken hangen af van de specifieke hartdefecten die ermee geassocieerd zijn, zoals enkelvoudige of gecorrigeerde transpositie van de grote slagaders en ventrikelseptumdefecten."},"pt":{"name":"Coração cruzado","synonyms":["Comunicações auriculo-ventriculares cruzadas"]},"pl":{"name":"Wada serca typu criss-cross heart","synonyms":["Criss-cross atrioventricular relationships","Superoinferior ventricles","Twisted atrioventricular connections"],"definition":"Serce typu criss-cross heart (CCH) to wada serca, w której napływy z dwóch komór krzyżują się z powodu skręcenia serca wokół jego osi długiej. Objawy kliniczne zależą od rodzaju wad towarzyszących, na przykład prostego lub skorygowanego przełożenia dużych naczyń i wad przegrody międzykomorowej."},"cs":{"name":"Crisscross srdce","synonyms":["Criss-cross vztah síní a komor","Superoinferiorní poloha komor","Zkřížené spojení síní a komor"]},"uk":{"name":"Criss-cross heart","synonyms":["Перехресні атріовентрикулярні звязки","Наднижній шлуночок","Перекручені атріовентрикулярні зєднання"],"definition":"Criss cross heart (CCH) is a cardiac malformation where the inflow streams of the two ventricles cross due to twisting of the heart about its major axis. The clinical features depend on the particular cardiac defects associated, like simple or corrected transposition of the great arteries and ventricular septal defects."},"zh":{"name":"交叉心脏","synonyms":["房室传导交叉"]},"tr":{"name":"Çapraz çizgili kalp","synonyms":["Süper-inferior ventriküller","Bükülmüş atriyoventriküler bağlantılar","Çaprazlama-ters atriyoventriküler ilişkiler"]}},"xrefs":[{"source":"ICD-10","code":"Q24.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA81","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"856695997"},{"source":"MONDO","code":"0015449","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D003420","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0010334","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":98730,"name":"OBSOLETE: Atrioventricular discordance","status":"inactive"}],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":88991,"name":"Rare congenital heart disease","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Prevalence at birth","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.8,"area":"Europe","status":"Validated","source":"ISBN:9781416023906[OTHER]_22555779[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0030680","label":"Abnormal cardiovascular system morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000961","label":"Cyanosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001633","label":"Abnormal mitral valve morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011968","label":"Feeding difficulties","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001718","label":"Mitral stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001642","label":"Pulmonic stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004381","label":"Supravalvular aortic stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001669","label":"Transposition of the great arteries","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010446","label":"Tricuspid stenosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001629","label":"Ventricular septal defect","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001999","label":"Abnormal facial shape","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000765","label":"Abnormal thorax morphology","frequency":"Occasional (29-5%)","criteria":null}]}}