{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,26]],"date-time":"2026-07-26T06:16:38Z","timestamp":1785046598730,"version":"3.55.0"},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"3","license":[{"start":{"date-parts":[[2025,3,1]],"date-time":"2025-03-01T00:00:00Z","timestamp":1740787200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Research Institute through the Victorian Government"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,3,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>OBJECTIVES<\/jats:title>\n                  <jats:p>The impact of conotruncal anomalies (CTAs), including tetralogy of Fallot, truncus arteriosus, ventriculo-arterial discordance, double-outlet right ventricle and interrupted aortic arch type B, on long-term outcomes remains poorly described in the Fontan cohort. We sought to review the outcomes of Fontan patients with CTAs in Australia and New Zealand.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>METHODS<\/jats:title>\n                  <jats:p>We reviewed the data from 1835 patients who underwent a Fontan operation between 1975 and 2023 from the Australia and New Zealand Fontan Registry.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>RESULTS<\/jats:title>\n                  <jats:p>CTAs occurred in 895 patients (49%), including D-TGA (Transposition of Great Arteries) (n\u2009=\u2009476), double-outlet right ventricle (n\u2009=\u2009360) and L-TGA (n\u2009=\u2009170). Patients with CTAs had more heterotaxy syndrome (11.3% vs 6.8%, P\u2009&amp;lt;\u20090.001) and less left-ventricular dominance (53% vs 60%, P\u2009=\u20090.011). Median follow-up was 11.7\u2009years (IQR: 5.3\u201320.3\u2009years). Overall transplant-free survival and freedom from Fontan failure was 84% (95% confidence interval: 81\u201387%) and 72% (95% confidence interval: 68\u201375%) at 20\u2009years, respectively. No difference was demonstrated in survival or freedom from Fontan failure between patients with or without CTAs (P\u2009=\u20090.50 and P\u2009=\u20090.83). Pacemaker implantation was more common in patients with CTAs (11.2% vs 8.3%, P\u2009=\u20090.038). Overall, 45 patients underwent outflow tract reinterventions, including semilunar valves, \u2018after\u2019 Fontan operation. Freedom from these reinterventions was 95% (95% confidence interval: 93\u201396%) at 30\u2009years and was higher in patients with CTAs (P\u2009&amp;lt;\u20090.001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>CONCLUSIONS<\/jats:title>\n                  <jats:p>Patients with CTAs did not have a demonstrable difference in long-term survival and freedom from Fontan failure to other patients undergoing Fontan operation. Patients with CTAs have higher incidence of outflow tract reinterventions, including semilunar valves, and higher rate of pacemaker implantation.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ejcts\/ezaf070","type":"journal-article","created":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T07:40:01Z","timestamp":1741678801000},"source":"Crossref","is-referenced-by-count":7,"title":["Outcomes of Fontan operation in patients with conotruncal anomalies from Australia and New Zealand Fontan Registry: a review of 1835 patients"],"prefix":"10.1093","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2016-7057","authenticated-orcid":false,"given":"Michael","family":"Daley","sequence":"first","affiliation":[{"name":"Department of Cardiac Surgery, Royal Children\u2019s Hospital , Melbourne,","place":["Australia"]},{"name":"Department of Paediatrics, University of Melbourne , Melbourne,","place":["Australia"]},{"name":"Heart Research Group, Murdoch Children\u2019s Research Institute , Melbourne,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael","family":"Cordina","sequence":"additional","affiliation":[{"name":"Sydney Medical School, University of Sydney , Sydney,","place":["Australia"]},{"name":"Department of Cardiology, Royal Prince Alfred Hospital , Sydney,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nelson","family":"Alphonso","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, Queensland Children\u2019s Hospital , Brisbane,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ajay","family":"Iyengar","sequence":"additional","affiliation":[{"name":"Green Lane Paediatric and Congenital Cardiac Service, Starship Children\u2019s Hospital , Auckland,","place":["New Zealand"]},{"name":"Department of Surgery, Auckland University , Auckland,","place":["New Zealand"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip A","family":"Roberts","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Children\u2019s Hospital at Westmead , Sydney,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry","family":"Robertson","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Women\u2019s and Children\u2019s Hospital , Adelaide,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Andrews","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, Perth Children\u2019s Hospital , Perth,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"d\u2019Udekem","sequence":"additional","affiliation":[{"name":"The Division of Cardiovascular Surgery, Children\u2019s National Heart Institute, Children\u2019s National Hospital , Washington, DC,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9981-1123","authenticated-orcid":false,"given":"Igor E","family":"Konstantinov","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, Royal Children\u2019s Hospital , Melbourne,","place":["Australia"]},{"name":"Department of Paediatrics, University of Melbourne , Melbourne,","place":["Australia"]},{"name":"Heart Research Group, Murdoch Children\u2019s Research Institute , Melbourne,","place":["Australia"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,3,11]]},"reference":[{"key":"2025032208012117200_ezaf070-B1","doi-asserted-by":"crossref","DOI":"10.1007\/978-3-319-23057-3","volume-title":"Surgery of Conotruncal 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