{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T03:03:44Z","timestamp":1768878224713,"version":"3.49.0"},"reference-count":23,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2022,8,31]],"date-time":"2022-08-31T00:00:00Z","timestamp":1661904000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100003725","name":"National Research Foundation of Korea (NRF)","doi-asserted-by":"publisher","award":["NRF2020R1F1A1076452"],"award-info":[{"award-number":["NRF2020R1F1A1076452"]}],"id":[{"id":"10.13039\/501100003725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: In the present study, the population prevalence and postoperative morbidity and mortality in Down syndrome patients who underwent total correction for congenital heart disease were investigated using data from a large national cohort. Methods: Retrospective administrative data from 2,395,966 participants born between 2008 and 2012 were acquired from the National Investigation of Birth Cohort in Korea. Among Down syndrome patients, 58.3% had congenital heart disease and 32.3% underwent total correction. Propensity score matching (maximum 1:1) and stabilized inverse probability treatment weighting (IPTW) were performed for each group (153 Down syndrome patients and 4482 non-Down syndrome patients). Results: T late mortality rate was significantly higher in the Down syndrome group than in the non-Down syndrome group (8.1% vs. 3.8%). No differences were observed in postoperative heart failure and arrhythmias, but pulmonary hypertension was significantly greater in the Down syndrome group than in the non-Down syndrome group (26.9% vs. 7.0%). The length of hospitalization was longer in the Down syndrome group than in the non-Down syndrome group (14 days vs. 11 days; interquartile range (IQR): 10\u201325 vs. 6\u201319; p &lt; 0.0001). After total correction, readmission frequency for any reason was minimally but statistically significantly higher in the Down syndrome group compared to the non-Down syndrome group (5 times vs. 5 times; IQR: 3\u20138 vs. 4\u20139; p &lt; 0.0001). However, the number of emergency room visits was minimally but significantly lower in the Down syndrome group compared to the non-Down syndrome group (2 visits vs. 2 visits (IQR): 2\u20137 vs. 1\u20134; p = 0.016). Conclusions: Down syndrome patients with congenital heart disease undergoing total correction showed pulmonary hypertension after surgery, longer length of hospitalization, frequent hospitalization after surgery, and a higher rate of late mortality.<\/jats:p>","DOI":"10.3390\/children9091329","type":"journal-article","created":{"date-parts":[[2022,8,31]],"date-time":"2022-08-31T23:53:21Z","timestamp":1661990001000},"page":"1329","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Long-Term Observational Outcomes after Total Correction of Congenital Heart Disease in Korean Patients with Down Syndrome: A National Cohort Study"],"prefix":"10.3390","volume":"9","author":[{"given":"Ji Hee","family":"Kwak","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul 03181, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5632-5208","authenticated-orcid":false,"given":"Seung Won","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Data Science, Sejong University College of Software Convergence, Seoul 05006, Korea"},{"name":"Department of Precision Medicine, Sungkyunkwan University School of Medicine, Suwon 16419, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0459-7655","authenticated-orcid":false,"given":"Hye Ryeong","family":"Cha","sequence":"additional","affiliation":[{"name":"Department of Data Science, Sejong University College of Software Convergence, Seoul 05006, Korea"},{"name":"Department of Computer Science and Engineering, Sungkyunkwan University, Suwon 16419, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"June","family":"Huh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I-Seok","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tae-Gook","family":"Jun","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ji-Hyuk","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9077-5779","authenticated-orcid":false,"given":"Man Yong","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, CHA Bundang Medical Center, CHA University School of Medicine, 59, Yatap-ro, Bundang-gu, Seongnam-si 13496, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9660-5949","authenticated-orcid":false,"given":"Jinyoung","family":"Song","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Adult Congenital Heart Clinic, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea"},{"name":"Department of Pediatrics, Samsung Medical Center, Heart Vascular Stroke Institute, Grown-Up Congenital Heart Clinic, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e20160123","DOI":"10.1542\/peds.2016-0123","article-title":"Trends in Congenital Heart Defects in Infants with Down Syndrome","volume":"138","author":"Carr","year":"2016","journal-title":"Pediatrics"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1111\/apa.14223","article-title":"The five-year survival of children with Down syndrome in Norway 1994\u20132009 differed by associated congenital heart defects and extracardiac malformations","volume":"107","author":"Brodwall","year":"2018","journal-title":"Acta Paediatr."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1111\/chd.12521","article-title":"Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome","volume":"12","year":"2017","journal-title":"Congenit. 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Surg."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/9\/1329\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:21:10Z","timestamp":1760142070000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/9\/1329"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,31]]},"references-count":23,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2022,9]]}},"alternative-id":["children9091329"],"URL":"https:\/\/doi.org\/10.3390\/children9091329","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,31]]}}}