{"orpha":1666,"name":"Dextrocardia","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, congenital, non-syndromic, developmental defect during embryogenesis characterized by positioning of the heart in the right hemithorax, with the base and apex of the heart pointing caudally and to the right, due to abnormalities of embryologic origin that are intrinsic to the heart itself. Situs inversus or situs solitus may be associated, with extracardiac visceral transposition anomalies usually present in the former case and additional cardiac defects (e.g. septal defects, transposition of the great arteries, double-outlet right ventricle, anomalous pulmonary venous return, tetralogy of Fallot) frequently observed in both cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1666/","orphanet_url":"https://www.orpha.net/en/disease/detail/1666","names":{"fr":{"name":"Dextrocardie","definition":"Anomalie rare, congénitale et non syndromique du développement embryonnaire, caractérisée par la présence du coeur dans l'hémithorax droit (avec la base et l'apex du coeur orientés vers la droite et sa partie caudale) due à des malformations intrinsèques au coeur qui surviennent au cours du développement intra-utérin. Un situs inversus ou un situs solitus peuvent être associés. Des anomalies extracardiaques de transposition viscérale sont généralement présentes en cas de situs inversus, et d'autres malformations cardiaques (par exemple anomalies du septum, transposition des gros vaisseaux, ventricule droit à double issue, retour veineux pulmonaire anormal, tétralogie de Fallot) sont fréquemment observées dans les deux cas."},"de":{"name":"Dextrokardie","definition":"Ein seltener, nicht-syndromaler genetischer Defekt, der durch eine Entwicklungsanomalie des Embryos verursacht wird und durch die Positionierung des Herzens im rechten Hemithorax gekennzeichnet ist, wobei die Basis und die Spitze nach kaudal rechts gerichtet sind. Situs inversus oder Situs solitus können assoziiert sein, wobei im ersten Fall in der Regel eine extrakardiale viszerale Transposition vorliegt und in beiden Fällen häufig begleitende Herzfehler beobachtet werden (z. B. Septumdefekte, Transposition der großen Arterien, rechter Ventrikel mit doppeltem Abfluss, abnormer pulmonalvenöser Rückfluss, Fallot-Tetralogie)."},"es":{"name":"Dextrocardia","definition":"Es un defecto no sindrómico del desarrollo embrionario, congénito y poco frecuente, caracterizado por la localización del corazón en el hemitórax derecho, con la base y el ápex cardíacos orientados de forma caudal y hacia la derecha debido a anomalías de origen embrionario intrínsecas al propio corazón. Este trastorno puede asociar situs inversus o situs solitus, que suele ir acompañado, en el primer caso, de anomalías extracardíacas de transposición visceral y de defectos cardíacos adicionales (p. ej. comunicación interauricular, transposición de las grandes arterias, doble salida del ventrículo derecho, drenaje venoso pulmonar anómalo, tetralogía de Fallot) observados con frecuencia en ambos casos."},"it":{"name":"Destrocardia","definition":"È un difetto genetico raro non sindromico di origine malformativa, causato da un'anomalia dello sviluppo dell'embrione, caratterizzata dal posizionamento del cuore nell'emitorace destro, con la base e l'apice rivolti caudalmente verso destra, è dovuto ad anomalie embrionali intrinseche al cuore. Può associarsi a situs inversus o situs solitus; nel primo caso il paziente di solito presenta anomalie extracardiache da trasposizione viscerale, mentre in entrambi i casi spesso sono presenti altre cardiopatie (ad es. difetti del setto, trasposizione delle grandi arterie, ventricolo destro a doppia uscita, ritorno venoso polmonare anomalo, tetralogia di Fallot)."},"nl":{"name":"Dextrocardie","definition":"Een zeldzaam, congenitaal, niet-syndromaal ontwikkelingsdefect tijdens de embryogenese, gekarakteriseerd door positionering van het hart in de rechter hemithorax en een hart met caudaal en naar rechts gerichte basis en apex, als gevolg van afwijkingen van embryologische oorsprong die intrinsiek zijn aan het hart. Situs inversus of situs solitus kan geassocieerd zijn, waarbij in het eerste geval meestal extracardiale viscerale transpositie aanwezig is, en waarbij in beide gevallen frequent bijkomende hartdefecten (e.g. septumdefecten, transpositie van de grote slagaders, dubbele uitstroom rechterventrikel, abnormale pulmonale veneuze terugstroom, tetralogie van Fallot) worden waargenomen."},"pt":{"name":"Dextrocardia"},"pl":{"name":"Dekstrokardia"},"cs":{"name":"Dextrokardie"},"uk":{"name":"Декстрокардія","definition":"A rare, congenital, non-syndromic, developmental defect during embryogenesis characterized by positioning of the heart in the right hemithorax, with the base and apex of the heart pointing caudally and to the right, due to abnormalities of embryologic origin that are intrinsic to the heart itself. Situs inversus or situs solitus may be associated, with extracardiac visceral transposition anomalies usually present in the former case and additional cardiac defects (e.g. septal defects, transposition of the great arteries, double-outlet right ventricle, anomalous pulmonary venous return, tetralogy of Fallot) frequently observed in both cases."},"zh":{"name":"右位心"},"tr":{"name":"Dekstrokardi"}},"xrefs":[{"source":"ICD-10","code":"Q24.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"LA80.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1472687600"},{"source":"MONDO","code":"0015661","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D003914","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0011813","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1827","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10012592","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":98716,"name":"Heart position anomaly","classifications":[147,148]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy","Neonatal"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2017-07-12","source":"25674744[PMID]_21861958[PMID]_17631088[PMID]_17515336[PMID]_15219529[PMID]_15219529[PMID]_13547381[PMID]","terms":[{"hpo":"HP:0001651","label":"Dextrocardia","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0003115","label":"Abnormal EKG","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001627","label":"Abnormal heart morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010872","label":"T-wave inversion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011603","label":"Congenital malformation of the great arteries","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001696","label":"Situs inversus totalis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002101","label":"Abnormal lung lobation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012210","label":"Abnormal renal morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012243","label":"Abnormal reproductive system morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011620","label":"Abnormality of abdominal situs","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011615","label":"Abnormality of pulmonary situs","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004414","label":"Abnormality of the pulmonary artery","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001743","label":"Abnormality of the spleen","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000069","label":"Abnormality of the ureter","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002566","label":"Intestinal malrotation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001760","label":"Abnormal foot morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000772","label":"Abnormal rib morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008771","label":"Aplasia/Hypoplasia of the ear","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001374","label":"Congenital hip dislocation","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001263","label":"Global developmental delay","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000238","label":"Hydrocephalus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002245","label":"Meckel diverticulum","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003006","label":"Neuroblastoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002594","label":"Pancreatic hypoplasia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000384","label":"Preauricular skin tag","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000465","label":"Webbed neck","frequency":"Very rare (<4-1%)","criteria":null}]}}