{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T17:25:47Z","timestamp":1787505947880,"version":"build-2736575974"},"reference-count":24,"publisher":"SAGE Publications","issue":"2","license":[{"start":{"date-parts":[[2011,4,1]],"date-time":"2011-04-01T00:00:00Z","timestamp":1301616000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["World J Pediatr Congenit Heart Surg"],"published-print":{"date-parts":[[2011,4]]},"abstract":"<jats:p>According to The International Society for Nomenclature of Pediatric and Congenital Heart Disease (ISNPCHD), \u201cHeterotaxy is synonymous with \u2018visceral heterotaxy\u2019 and \u2018heterotaxy syndrome\u2019. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as \u2018situs solitus\u2019, or patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as `situs inversus\u2019.\u201d or patients with complete mirror-image arrangement of the internal organs along the left\u2013right axis, also known as situs inversus. The purpose of this article is to review the data about heterotaxy in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database. The investigators examined all index operations in the STS Congenital Heart Surgery Database over 12 years from January 1, 1998 to December 31, 2009, inclusive. This analysis resulted in a cohort of 77\u2009153 total index operations. Of these, 1505 operations (1.95%) were performed in patients with heterotaxy. Of the 1505 index operations performed in patients with heterotaxy, 1144 were in patients with asplenia and 361 were in patients with polysplenia. In every STS -EACTS Congenital Heart Surgery Mortality Category, discharge mortality is higher in patients with heterotaxy compared with patients without heterotaxy (EACTS = European Association for Cardio-Thoracic Surgery). Discharge mortality after systemic to pulmonary artery shunt is 6.6% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 10.8% in single-ventricle patients with heterotaxy. Discharge mortality after Fontan is 1.8% in a cohort of all single-ventricle patients except those with heterotaxy, whereas it is 4.2% in single-ventricle patients with heterotaxy. The STS Congenital Heart Surgery Database is largest congenital heart surgery database in North America. This review of data from the STS Congenital Heart Surgery Database allows for unique documentation of practice patterns and outcomes. From this analysis, it is clear that heterotaxy is a challenging problem with increased discharge mortality in most subgroups.<\/jats:p>","DOI":"10.1177\/2150135110397670","type":"journal-article","created":{"date-parts":[[2011,4,13]],"date-time":"2011-04-13T17:46:36Z","timestamp":1302716796000},"page":"278-286","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":53,"title":["Heterotaxy"],"prefix":"10.1177","volume":"2","author":[{"given":"Jeffrey Phillip","family":"Jacobs","sequence":"first","affiliation":[{"name":"The Congenital Heart Institute of Florida (CHIF), All Children\u2019s Hospital and Children\u2019s Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates of Florida (CSAoF), Saint Petersburg and Tampa, FL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara K.","family":"Pasquali","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Duke University School of Medicine, and Duke Clinical Research Institute, Duke University Medical Center, Durham, NC, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David L. S.","family":"Morales","sequence":"additional","affiliation":[{"name":"Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, and Division of Congenital Heart Surgery, Texas Children\u2019s Hospital, Houston, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marshall Lewis","family":"Jacobs","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Foundation, Cleveland Clinic Lerner School of Medicine, Case Western Reserve University, Cleveland, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constantine","family":"Mavroudis","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Foundation, Cleveland Clinic Lerner School of Medicine, Case Western Reserve University, Cleveland, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul Jubeong","family":"Chai","sequence":"additional","affiliation":[{"name":"The Congenital Heart Institute of Florida (CHIF), All Children\u2019s Hospital and Children\u2019s Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates of Florida (CSAoF), Saint Petersburg and Tampa, FL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christo I.","family":"Tchervenkov","sequence":"additional","affiliation":[{"name":"The Montreal Children\u2019s Hospital of the McGill University Health Centre, Montreal, Quebec, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francois G.","family":"Lacour-Gayet","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital at Montefiore, New York, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"Hal","family":"Walters","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Michigan, Wayne State University School of Medicine, Detroit, MI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James Anthony","family":"Quintessenza","sequence":"additional","affiliation":[{"name":"The Congenital Heart Institute of Florida (CHIF), All Children\u2019s Hospital and Children\u2019s Hospital of Tampa, University of South Florida College of Medicine, Cardiac Surgical Associates of Florida (CSAoF), Saint Petersburg and Tampa, FL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2011,4,13]]},"reference":[{"key":"bibr1-2150135110397670","volume-title":"Executive Summary: The Society of Thoracic Surgeons Congenital Heart Surgery Database\u2014Twelfth Harvest\u2014(January 1, 2006\u2013December 31, 2009)","author":"Jacobs JP","year":"2010"},{"key":"bibr2-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951108002813"},{"key":"bibr3-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2010.02.003"},{"issue":"2","key":"bibr4-2150135110397670","first-page":"1","volume":"17","author":"Jacobs JP","year":"2007","journal-title":"Cardiol Young"},{"key":"bibr5-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1177\/2150135110361504"},{"key":"bibr6-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1177\/2150135110381392"},{"key":"bibr7-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951108002795"},{"key":"bibr8-2150135110397670","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(99)01278-3"},{"key":"bibr9-2150135110397670","unstructured":"STS Congenital Heart Surgery Database data specifications version 3.0. http:\/\/www.sts.org\/documents\/pdf\/CongenitalDataSpecificationsV3_0_20090904.pdf. 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