{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T18:10:48Z","timestamp":1787508648589,"version":"build-2736575974"},"reference-count":11,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2022,6,25]],"date-time":"2022-06-25T00:00:00Z","timestamp":1656115200000},"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":[[2022,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Patients with heterotaxy syndromes (right and left atrial isomerism) are at high risk of poor outcomes after single-ventricle palliation. However, the long-term outcomes and specific parameters associated with poor outcomes are incompletely understood.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective review was performed of all patients with atrial isomerism who had a Fontan at our institution from 1973 to 2020. Standard demographic, as well as pre-, peri-, and postoperative parameters were collected. Features and outcomes of patients with polysplenia were compared to asplenia. Outcomes were analyzed for effect during 4 eras: (1: 1973-1984; n\u2009=\u200927), (2: 1985-1994; n\u2009=\u200993), (3: 1995-2004; n\u2009=\u200928), and (4: 2005-2020; n\u2009=\u200910).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Of the 1176 patients who had a Fontan operation, 158 (14%) had a heterotaxy syndrome. The median age at the time of Fontan was 8 (9) years. Early mortality was 20% and was greater in patients with asplenia compared to polysplenia (27% vs. 12%, p\u2009=\u2009.019). But it substantially improved over time (61% in era 1 vs. 7%-10% in the more recent eras ( p\u2009&lt;\u2009.001)), as did transplant-free survival (22% at 10 years in era 1 vs. 88% in era 4, p\u2009&lt;\u2009.001). Transplant-free survival was significantly lower in patients with asplenia versus those with polysplenia ( p\u2009=\u2009.014), and patients with heterotaxy had lower survival than nonheterotaxy ( p\u2009=\u2009.01). This was largely due to the asplenia group ( p\u2009&lt;\u2009.001) (hazard ratio\u2009=\u20093.05, p\u2009=\u2009.007).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>After Fontan operation, patients with heterotaxy, particularly asplenia, continue to demonstrate worse transplant-free survival than nonheterotaxy patients. Early mortality and long-term transplant-free survival have improved in more recent eras.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/21501351221099944","type":"journal-article","created":{"date-parts":[[2022,6,25]],"date-time":"2022-06-25T04:05:23Z","timestamp":1656129923000},"page":"436-442","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["Fontan Palliation in Patients with Heterotaxy Syndrome: A Five Decade Experience"],"prefix":"10.1177","volume":"13","author":[{"given":"Elizabeth H.","family":"Stephens","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriel","family":"Graham","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2664-1290","authenticated-orcid":false,"given":"Joseph A.","family":"Dearani","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talha","family":"Niaz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Cetta","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA"},{"name":"Department of Cardiovascular Diseases Rochester, Mayo Clinic, MN, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2022,6,25]]},"reference":[{"key":"bibr1-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1177\/2150135110397670"},{"key":"bibr2-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1177\/2150135110396908"},{"key":"bibr3-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(92)90024-H"},{"key":"bibr4-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2006.07.053"},{"key":"bibr5-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(02)04124-3"},{"key":"bibr6-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2019.11.015"},{"key":"bibr7-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2006.04.082"},{"key":"bibr8-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1177\/021849230601400314"},{"key":"bibr9-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951107001151"},{"key":"bibr10-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2018.12.025"},{"key":"bibr11-21501351221099944","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2020.09.028"}],"container-title":["World Journal for Pediatric and Congenital Heart Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/21501351221099944","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/21501351221099944","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/21501351221099944","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T23:24:43Z","timestamp":1777591483000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/21501351221099944"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,25]]},"references-count":11,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,7]]}},"alternative-id":["10.1177\/21501351221099944"],"URL":"https:\/\/doi.org\/10.1177\/21501351221099944","relation":{},"ISSN":["2150-1351","2150-136X"],"issn-type":[{"value":"2150-1351","type":"print"},{"value":"2150-136X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,25]]}}}