{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T23:23:21Z","timestamp":1792193001159,"version":"4.4.0"},"reference-count":37,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,8,5]],"date-time":"2024-08-05T00:00:00Z","timestamp":1722816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Obstet Gynecol Scand"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>The prevalence of Down syndrome (DS) is approximately 1 per 1000 births and is influenced by increasing maternal age over the last few decades. DS is strongly associated with congenital heart defects (CHDs), especially atrioventricular septal defect (AVSD). Our objectives were to investigate the prevalence of live\u2010born infants with DS having a severe CHD in the Norwegian population over the last 20\u2009years and compare outcomes in infants with AVSD with and without DS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and Methods<\/jats:title><jats:p>Information on all births from January 1, 2000 to December 31, 2019 was obtained from the Medical Birth Registry of Norway. We also obtained data on all infants with severe CHDs in Norway registered in Oslo University Hospital's Clinical Registry for Congenital Heart Defects during 2000\u20132019 and accessed individual\u2010level patient data from the electronic hospital records of selected cases. Infants with AVSD and DS were compared to infants with AVSD without chromosomal defects. Crude and adjusted odds ratios (ORs) of infant mortality and need for surgery during the first year of life, with associated 95% confidence intervals (CIs), were estimated by logistic regression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 1\u2009177\u2009926 infants were live\u2010born in Norway during the study period. Among these, 1456 (0.1%) had DS. The prevalence of infants with DS having a severe CHDs was relatively stable, with a mean of 17 cases per year. The most common CHD associated with DS was AVSD (44.4%). Infants with AVSD and DS were more likely to have cardiac intervention during their first year of life compared to infants with AVSD without chromosomal defects (adjusted OR [aOR]: 2.52; 95% CI 1.27, 4.98). However, we observed no difference in infant mortality during first year of life between the two groups (aOR: 1.08; 95% CI 0.43, 2.70).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The prevalence of live\u2010born infants with severe CHDs and DS has been stable in Norway across 20\u2009years. Infants with AVSD and DS did not have higher risk of mortality during their first year of life compared to infants with AVSD without chromosomal defects, despite a higher risk of operative intervention.<\/jats:p><\/jats:sec>","DOI":"10.1111\/aogs.14932","type":"journal-article","created":{"date-parts":[[2024,8,6]],"date-time":"2024-08-06T05:41:09Z","timestamp":1722922869000},"page":"2024-2030","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Down syndrome and associated atrioventricular septal defects in a nationwide Norwegian cohort: Prevalence, time trends, and outcomes"],"prefix":"10.1111","volume":"103","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-2832-4226","authenticated-orcid":false,"given":"Cecilie Bryn","family":"Nordklev","sequence":"first","affiliation":[{"name":"Department of Fetal Medicine Oslo University Hospital  Oslo Norway"},{"name":"Faculty of Medicine University of Oslo  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1913-6445","authenticated-orcid":false,"given":"Ola","family":"Gjesdal","sequence":"additional","affiliation":[{"name":"Department of Cardiology Oslo University Hospital  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2606-0648","authenticated-orcid":false,"given":"Nina","family":"Gunnes","sequence":"additional","affiliation":[{"name":"Norwegian Research Center for Women's Health Oslo University Hospital  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4745-5267","authenticated-orcid":false,"given":"Henrik","family":"Holmstr\u00f8m","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Oslo  Oslo Norway"},{"name":"Department of Pediatric Cardiology Oslo University Hospital  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nils\u2010Halvdan","family":"Morken","sequence":"additional","affiliation":[{"name":"Department of Clinical Science University of Bergen  Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4681-4774","authenticated-orcid":false,"given":"Siri","family":"Vangen","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of Oslo  Oslo Norway"},{"name":"Norwegian Research Center for Women's Health Oslo University Hospital  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3264-3302","authenticated-orcid":false,"given":"Vasilis","family":"Sitras","sequence":"additional","affiliation":[{"name":"Department of Fetal Medicine Oslo University Hospital  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,5]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdra.20666"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-010-1253-0"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/bdra.20393"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19981116)80:3<213::AID-AJMG6>3.0.CO;2-8"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e3181634867"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980605)77:5<431::AID-AJMG15>3.0.CO;2-J"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36780"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.a2547"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2016-0123"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/uog.24888"},{"key":"e_1_2_11_12_1","unstructured":"Lov om humanmedisinsk bruk av bioteknologi m.m. 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