{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T01:49:50Z","timestamp":1789523390508,"version":"build-2803163510"},"reference-count":17,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2025,2,26]],"date-time":"2025-02-26T00:00:00Z","timestamp":1740528000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951125000435_as1\"><jats:title>Objective:<\/jats:title><jats:p>This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951125000435_as2\"><jats:title>Methods:<\/jats:title><jats:p>This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951125000435_as3\"><jats:title>Results:<\/jats:title><jats:p>A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and\/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5\/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).<\/jats:p><\/jats:sec><jats:sec id=\"S1047951125000435_as4\"><jats:title>Conclusions:<\/jats:title><jats:p>Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951125000435","type":"journal-article","created":{"date-parts":[[2025,2,26]],"date-time":"2025-02-26T08:35:53Z","timestamp":1740558953000},"page":"722-725","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":2,"title":["Kabuki patients with CHDs: outcomes after cardiac surgery"],"prefix":"10.1017","volume":"35","author":[{"given":"Regina W.","family":"Lam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victoria","family":"Haro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Tabbutt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroo","family":"Kinami","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V. Mohan","family":"Reddy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5833-2572","authenticated-orcid":false,"given":"Minso","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"56","published-online":{"date-parts":[[2025,2,26]]},"reference":[{"key":"S1047951125000435_ref1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320310312"},{"key":"S1047951125000435_ref9","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.38417"},{"key":"S1047951125000435_ref14","doi-asserted-by":"publisher","DOI":"10.1053\/j.semtcvs.2018.12.004"},{"key":"S1047951125000435_ref3","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1265"},{"key":"S1047951125000435_ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2005.09.033"},{"key":"S1047951125000435_ref13","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-013-0766-5"},{"key":"S1047951125000435_ref10","doi-asserted-by":"publisher","DOI":"10.5603\/CJ.2013.0023"},{"key":"S1047951125000435_ref5","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2004.00348.x"},{"key":"S1047951125000435_ref15","first-page":"77","article-title":"Robotic surgery for atrial septal defect closure in a case of Kabuki syndrome","volume":"45","author":"Onan","year":"2017","journal-title":"Turk Kardiyol Dern Ars"},{"key":"S1047951125000435_ref11","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-017-1717-3"},{"key":"S1047951125000435_ref4","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddv180"},{"key":"S1047951125000435_ref2","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2018-105625"},{"key":"S1047951125000435_ref16","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2020.01.036"},{"key":"S1047951125000435_ref17","doi-asserted-by":"publisher","DOI":"10.1111\/j.1747-0803.2007.00136.x"},{"key":"S1047951125000435_ref6","doi-asserted-by":"publisher","DOI":"10.32604\/CHD.2021.014409"},{"key":"S1047951125000435_ref7","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(99)70207-6"},{"key":"S1047951125000435_ref8","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19981204)80:4<362::AID-AJMG11>3.0.CO;2-W"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951125000435","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T04:02:32Z","timestamp":1743566552000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951125000435\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,26]]},"references-count":17,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2025,4]]}},"alternative-id":["S1047951125000435"],"URL":"https:\/\/doi.org\/10.1017\/s1047951125000435","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"value":"1047-9511","type":"print"},{"value":"1467-1107","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,2,26]]},"assertion":[{"value":"\u00a9 The Author(s), 2025. Published by Cambridge University Press","name":"copyright","label":"Copyright","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This is an Open Access article, distributed under the terms of the Creative Commons Attribution licence (https:\/\/creativecommons.org\/licenses\/by\/4.0\/), which permits unrestricted re-use, distribution and reproduction, provided the original article is properly cited.","name":"license","label":"License","group":{"name":"copyright_and_licensing","label":"Copyright and Licensing"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}