{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T21:58:45Z","timestamp":1747173525522,"version":"3.40.5"},"reference-count":14,"publisher":"Cambridge University Press (CUP)","issue":"7","license":[{"start":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T00:00:00Z","timestamp":1627516800000},"content-version":"unspecified","delay-in-days":28,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The International Pediatric and Congenital Cardiac Code (IPCCC) states that<jats:italic>visceral heterotaxy<\/jats:italic>is defined as \u201ca congenital malformation in which the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, in congenital cardiology, heterotaxy syndrome does not include patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as \u201ctotal mirror imagery\u201d or \u201csitus inversus totalis\u201d.\u201d [<jats:uri xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"https:\/\/www.ipccc.net\">www.ipccc.net<\/jats:uri>]<\/jats:p><jats:p>In patients with heterotaxy, it is important to describe both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. Particular attention is required for the venoatrial connections, since these are so often abnormal. The relationship and arrangement of the remaining thoraco-abdominal organs, including the lungs, the spleen, the liver, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. Therefore, in patients with heterotaxy, it is important to describe each thoracic and abdominal organ independently.<\/jats:p>","DOI":"10.1017\/s1047951121002961","type":"journal-article","created":{"date-parts":[[2021,7,29]],"date-time":"2021-07-29T10:19:02Z","timestamp":1627553942000},"page":"1200-1201","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":2,"title":["Heterotaxy: fluctuat nec mergitur"],"prefix":"10.1017","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4024-8599","authenticated-orcid":false,"given":"Lucile","family":"Houyel","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3360-9341","authenticated-orcid":false,"given":"Anne","family":"Moreau de Bellaing","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damien","family":"Bonnet","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2021,7,29]]},"reference":[{"journal-title":"Cardiol Young","article-title":"Abnormal origin of the left pulmonary artery from the descending aorta and heterotaxy syndrome: an undescribed phenotypic association","author":"Moreau de Bellaing","key":"S1047951121002961_ref1"},{"key":"S1047951121002961_ref11","doi-asserted-by":"publisher","DOI":"10.1111\/joa.13378"},{"key":"S1047951121002961_ref3","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951107001138"},{"key":"S1047951121002961_ref5","unstructured":"5. Jacobs, JP , Franklin, RCG , B\u00e9land, MJ , et\u00a0al. Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature \u2013 The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). Cardiol Young. This issue."},{"key":"S1047951121002961_ref6","unstructured":"6. Jacobs, JP , Franklin, RCG , B\u00e9land, MJ , et\u00a0al. Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature \u2013 The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). World Journal for Pediatric and Congenital Heart Surgery. July 2021."},{"key":"S1047951121002961_ref7","doi-asserted-by":"crossref","first-page":"e006917","DOI":"10.1161\/CIRCIMAGING.117.006917","article-title":"Disharmonious patterns of heterotaxy and isomerism: how often are the classic patterns breached?","volume":"11","author":"Yim","year":"2018","journal-title":"Cardiovasc Imag"},{"key":"S1047951121002961_ref8","doi-asserted-by":"publisher","DOI":"10.1017\/S104795110700114X"},{"journal-title":"Cardiol Young","article-title":"Whither heterotaxy?","author":"Anderson","key":"S1047951121002961_ref2"},{"key":"S1047951121002961_ref12","unstructured":"12. Anonymous. A general and particular History of Anomalies of Organization in Man and Animals, comprising researches into the Characters, Classification, etc. of Monstrosities. By M. Isidore Geoffroy St Hilaire, M.D., etc. etc. Paris, 1832-36. 3 Vols. 8vo, with an Atlas. Br Foreign Med Rev 1839; VIII, n\u00b0XV: 1\u201339."},{"key":"S1047951121002961_ref13","unstructured":"13. Vierordt, H. Die angeborenen Herzkrankheiten. In Nothnagel\u2019s Spec Path Therapie 1-225, 1898."},{"key":"S1047951121002961_ref14","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.63.2.122"},{"key":"S1047951121002961_ref4","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951117002244"},{"key":"S1047951121002961_ref9","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36695"},{"key":"S1047951121002961_ref10","first-page":"4","article-title":"The segmental approach to diagnosis in congenital heart disease","volume":"8","author":"Van Praagh","year":"1972","journal-title":"Birth Defects Orig Artic Ser"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951121002961","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,1,5]],"date-time":"2023-01-05T20:56:31Z","timestamp":1672952191000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951121002961\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,7]]},"references-count":14,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2021,7]]}},"alternative-id":["S1047951121002961"],"URL":"https:\/\/doi.org\/10.1017\/s1047951121002961","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"type":"print","value":"1047-9511"},{"type":"electronic","value":"1467-1107"}],"subject":[],"published":{"date-parts":[[2021,7]]},"assertion":[{"value":"\u00a9 The Author(s), 2021. 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