{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T23:18:30Z","timestamp":1787008710483,"version":"build-2736575974"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2022,12,2]],"date-time":"2022-12-02T00:00:00Z","timestamp":1669939200000},"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":[[2022,12,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p\/>\n                  <jats:sec>\n                    <jats:title>OBJECTIVES<\/jats:title>\n                    <jats:p>Children with Alagille syndrome often have complex forms of congenital heart defects with the majority having peripheral pulmonary artery stenosis (PPAS) and pulmonary valve atresia (PA) or pulmonary valve stenosis. Children with Alagille syndrome also have variable amounts of liver dysfunction. The purpose of this study was to evaluate the impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>This was a retrospective review of 69 patients with Alagille syndrome who underwent congenital heart surgery at our institution. The underlying diagnoses included PPAS (n\u2009=\u200929), tetralogy of Fallot with PPAS (n\u2009=\u200914), tetralogy with PA (n\u2009=\u20093), PA with ventricular septal defect and major aortopulmonary collateral arteries (n\u2009=\u200921) and one each with D-transposition and supravalvar aortic stenosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>The median age at surgery was 16\u2009months (range 0\u2013228\u2009months). Procedures performed included PPAS repair (n\u2009=\u200943), tetralogy with PA repair (n\u2009=\u20093), unifocalization procedures (n\u2009=\u200921) and other (n\u2009=\u20092). Forty-two (61%) patients had mild or no liver dysfunction, while 26 (38%) had moderate or severe liver dysfunction. The median cardiopulmonary bypass time was 345\u2009min (341 with liver dysfunction, 345 without liver dysfunction). There were a total of 8 operative (12%) deaths and 3 late (4%) deaths. Six operative and 2 late deaths occurred in patients with liver dysfunction (combined 30.7%) versus 2 operative and 1 late death (combined 7.1%) for patients without liver dysfunction (P\u2009&amp;lt;\u20090.05).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>These results suggest that liver dysfunction has a profound impact on survival in children with Alagille syndrome undergoing congenital heart surgery.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejcts\/ezac553","type":"journal-article","created":{"date-parts":[[2022,12,1]],"date-time":"2022-12-01T17:52:23Z","timestamp":1669917143000},"source":"Crossref","is-referenced-by-count":6,"title":["Impact of liver dysfunction on outcomes in children with Alagille syndrome undergoing congenital heart surgery"],"prefix":"10.1093","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3312-4961","authenticated-orcid":false,"given":"Richard D","family":"Mainwaring","sequence":"first","affiliation":[{"name":"Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children\u2019s Hospital Stanford , Stanford, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L Mac","family":"Felmly","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children\u2019s Hospital Stanford , Stanford, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R Thomas","family":"Collins","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Stanford University School of Medicine, Lucile Packard Children\u2019s Hospital Stanford , Stanford, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank L","family":"Hanley","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiac Surgery, Stanford University School of Medicine, Lucile Packard Children\u2019s Hospital Stanford , Stanford, CA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,12,2]]},"reference":[{"key":"2022121922443805400_ezac553-B1","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S0022-3476(87)80153-1","article-title":"Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases","volume":"110","author":"Alagille","year":"1987","journal-title":"J Pediatr"},{"key":"2022121922443805400_ezac553-B2","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1002\/hep.510290331","article-title":"Features of Alagille syndrome in 92 patients: frequency and relation to 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