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Deaths were identified via record linkage to death registrations and five\u2010year survival was estimated using Kaplan\u2013Meier methods.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 8521 liveborn infants with <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content> of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five\u2010year survival rate was 92.8% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 92.2\u201393.3) and 83.2% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 79.0\u201387.4) for syndromes, 83.4% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 80.9\u201385.9) for multiple, 85.1% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 82.6\u201387.5) for chromosomal, 95.3% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 94.8\u201395.8) for isolated and 96.2% (95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 94.3\u201398.1) for non\u2010Q chapter anomalies. Five\u2010year survival for chromosomal, syndromes and sub\u2010groups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 77.5\u201081.4) than for term infants (95.8%; 95%<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>: 95.3\u201396.3).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Nine in ten infants with major <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content> survive up to five years, although there is variability in survival across <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content> groups. Survival of infants with major congenital anomalies has improved in recent years.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.14833","type":"journal-article","created":{"date-parts":[[2019,5,2]],"date-time":"2019-05-02T13:09:28Z","timestamp":1556802568000},"page":"2008-2018","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Five\u2010year survival of infants with major congenital anomalies: a registry based study"],"prefix":"10.1111","volume":"108","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6245-7159","authenticated-orcid":false,"given":"Francisco J.","family":"Schneuer","sequence":"first","affiliation":[{"name":"Child Population and Translational Health Research The Children's Hospital at Westmead Clinical School The University of Sydney Sydney New South Wales Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane 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