{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,27]],"date-time":"2025-09-27T22:07:39Z","timestamp":1759010859742,"version":"3.40.5"},"reference-count":20,"publisher":"Cambridge University Press (CUP)","issue":"8","license":[{"start":{"date-parts":[[2020,6,29]],"date-time":"2020-06-29T00:00:00Z","timestamp":1593388800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2020,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951120001791_as1\" sec-type=\"other\"><jats:title>Background:<\/jats:title><jats:p>The association of atrioventricular septal defect and transposition of the great arteries is very rare. As a rule, these patients have unbalanced ventricles. However, there have been no studies describing the results of single-ventricle palliation in these children.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001791_as2\" sec-type=\"other\"><jats:title>Methods:<\/jats:title><jats:p>All children who underwent surgery with a diagnosis of atrioventricular septal defect and transposition of the great arteries were included in the study. Data were obtained from medical records.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001791_as3\" sec-type=\"other\"><jats:title>Results:<\/jats:title><jats:p>A total of 38 patients with atrioventricular septal defect and transposition of the great arteries underwent single-ventricle palliation at the study institution between 1971 and 2016. The mean follow-up was 12.4 years (median: 14.6 years, range 2\u201343.3 years). Most children had unbalanced atrioventricular septal defect (94.7%, 36\/38). Survival was 67.6% (95% confidence interval [CI]: 50.0\u201380.2%) at 10 years and 57.8% (95% CI: 38.0\u201373.4%) at 20 years. By 10 years, 58.6% (95% CI: 40.8\u201372.7%) had progressed to Fontan completion, while 32.5% (95% CI: 18.2\u201347.6%) had died. In patients achieving Fontan completion, 20-year event-free survival was 73.3% (95% CI: 34.8\u201391.3%), while 5.0% (95% CI: 0.4\u201320.5%) had undergone cardiac transplantation and 21.7% (95% CI: 3.2\u201350.8%) had undergone takedown of the Fontan circulation. Freedom from atrioventricular valve surgery was 57.0% (95% CI: 37.2\u201372.7%) at 10 and 20 years.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951120001791_as4\" sec-type=\"other\"><jats:title>Conclusions:<\/jats:title><jats:p>The association of atrioventricular septal defect and transposition of the great arteries is very rare, and most of these children have unbalanced ventricles. Single-ventricle palliation results in 25-year overall survival of 50%. However, in patients, who had Fontan completion, survival was 75% at 25 years after Fontan operation.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951120001791","type":"journal-article","created":{"date-parts":[[2020,6,29]],"date-time":"2020-06-29T08:55:59Z","timestamp":1593420959000},"page":"1165-1170","source":"Crossref","is-referenced-by-count":3,"title":["Single-ventricle palliation in children with atrioventricular septal defect and transposition of the great arteries: 45 years of experience"],"prefix":"10.1017","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8330-3917","authenticated-orcid":false,"given":"Edward","family":"Buratto","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tyson A.","family":"Fricke","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xin Tao","family":"Ye","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johann","family":"Brink","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian P.","family":"Brizard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yves","family":"d\u2019Udekem","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Igor E.","family":"Konstantinov","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2020,6,29]]},"reference":[{"key":"S1047951120001791_ref20","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezx025"},{"key":"S1047951120001791_ref18","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezw018"},{"key":"S1047951120001791_ref16","first-page":"21","article-title":"Unbalanced atrioventricular septal defects","volume":"9","author":"Drinkwater","year":"1997","journal-title":"Semin Thorac Cardiovasc Surg"},{"key":"S1047951120001791_ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcts.2006.09.032"},{"key":"S1047951120001791_ref12","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2016.09.080"},{"volume-title":"Complete Transposition of Great Vessels","year":"1973","author":"Shaher","key":"S1047951120001791_ref9"},{"key":"S1047951120001791_ref1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezu286"},{"key":"S1047951120001791_ref2","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2017-312163"},{"key":"S1047951120001791_ref19","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2017.06.042"},{"key":"S1047951120001791_ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2016.09.051"},{"key":"S1047951120001791_ref10","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-005-0883-x"},{"key":"S1047951120001791_ref3","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezt444"},{"key":"S1047951120001791_ref13","doi-asserted-by":"publisher","DOI":"10.1016\/0003-4975(92)90671-P"},{"key":"S1047951120001791_ref4","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2012.03.019"},{"key":"S1047951120001791_ref6","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.135046"},{"key":"S1047951120001791_ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2016.01.085"},{"key":"S1047951120001791_ref8","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.61.6.1142"},{"key":"S1047951120001791_ref17","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-008-9376-z"},{"key":"S1047951120001791_ref11","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2015.04.111"},{"key":"S1047951120001791_ref5","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2015.05.011"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951120001791","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,8,13]],"date-time":"2020-08-13T09:35:26Z","timestamp":1597311326000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951120001791\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,29]]},"references-count":20,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2020,8]]}},"alternative-id":["S1047951120001791"],"URL":"https:\/\/doi.org\/10.1017\/s1047951120001791","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"type":"print","value":"1047-9511"},{"type":"electronic","value":"1467-1107"}],"subject":[],"published":{"date-parts":[[2020,6,29]]}}}