{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:47:06Z","timestamp":1787496426919,"version":"build-2736575974"},"reference-count":28,"publisher":"Oxford University Press (OUP)","issue":"6","license":[{"start":{"date-parts":[[2023,9,9]],"date-time":"2023-09-09T00:00:00Z","timestamp":1694217600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p\/>\n                  <jats:sec>\n                    <jats:title>OBJECTIVES<\/jats:title>\n                    <jats:p>The goal of this study was to determine the long-term surgical outcomes of patients with functional single ventricles associated with heterotaxy syndrome, risk factors for mortality and factors associated with Fontan stage completion.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>Overall, 279 patients with a functional single ventricle associated with heterotaxy syndrome who underwent an initial surgical procedure at our institute between 1978 and 2021 were grouped into 4 \u201ceras\u201d based on the surgical year during which the initial procedure was performed: era 1 (1978\u20131989, n\u2009=\u200971), era 2 (1990\u20131999, n\u2009=\u200998), era 3 (2000\u20132009, n\u2009=\u200964) and era 4 (2010\u20132021, n\u2009=\u200946). Neonatal surgery was more frequent in eras 3 and 4 than in eras 1 and 2.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>Overall, 228 patients had right atrial isomerism; 120 patients (43.0%) had a total anomalous pulmonary venous connection; and 58 patients (20.8%) underwent an initial procedure as neonates. Overall survival rates at 10, 20 and 30\u2009years after the initial procedure were 47.1%, 40.6% and 36.1%, respectively. Neonatal surgery (P\u2009&amp;lt;\u20090.001), total anomalous pulmonary venous connection repair at the initial procedure (P\u2009&amp;lt;\u20090.001) and early era (P\u2009&amp;lt;\u20090.001) were identified as risk factors for mortality, with the last 2 variables being negatively associated with Fontan stage completion (P\u2009&amp;lt;\u20090.001 for both).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>Although era had a favourable effect on survival, total anomalous pulmonary venous connection with intrinsic pulmonary vein obstruction was associated with both mortality and Fontan stage completion.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Clinical registration number<\/jats:title>\n                    <jats:p>R19092<\/jats:p>\n                  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