{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T16:19:06Z","timestamp":1785946746669,"version":"3.56.0"},"reference-count":30,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2021,1,6]],"date-time":"2021-01-06T00:00:00Z","timestamp":1609891200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005754","name":"Sahlgrenska Universitetssjukhuset","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005754","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005761","name":"Sahlgrenska Akademin","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005761","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>To study the long\u2010term outcome after surgery for pulmonary atresia and ventricular septal defect (PA\u2010VSD), and to determine association between the contribution of major aorto\u2010pulmonary collateral arteries (MAPCAs) to the pulmonary blood flow, comorbidity and cause of death.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients who had undergone surgery for PA\u2010VSD from January 1st 1994 to December 31st 2017 were studied retrospectively. Survival was cross\u2010checked against the Swedish National Population Register.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventy patients were identified, giving an incidence of 5.3 newborns per 100\u00a0000 live births. In 41 patients (59%) the pulmonary blood flow originated from a patent ductus arteriosus (PDA), while 29 patients (41%) had contribution of the pulmonary blood flow from MAPCAs. Extracardiac disease was found in 34 patients (49%), 16 of whom had 22q11\u2010microdeletion syndrome (23%). Survival at follow\u2010up was similar in patients with and without MAPCAs (72.4% vs. 75.6%, n.s.), with a median follow\u2010up time of 14.3\u00a0years (3.2\u201341.8\u00a0years). No difference was found in mortality in patients with or without any syndrome or extracardiac disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Long\u2010term survival did not differ between those with and without MAPCAs and no difference in mortality was seen in patients with and without concomitant extracardiac disease or any kind of syndrome.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.15732","type":"journal-article","created":{"date-parts":[[2020,12,23]],"date-time":"2020-12-23T06:31:48Z","timestamp":1608705108000},"page":"1610-1619","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Outcome after surgery for pulmonary atresia with ventricular septal defect, a long\u2010term follow\u2010up study"],"prefix":"10.1111","volume":"110","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4394-8427","authenticated-orcid":false,"given":"Erik","family":"Wiezell","sequence":"first","affiliation":[{"name":"Department of Paediatrics S\u00f6dra \u00c4lvsborg Hospital Bor\u00e5s Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janus","family":"F. Gudnason","sequence":"additional","affiliation":[{"name":"Children's Heart Center The Queen Silvia Children's Hospital Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mats","family":"Synnergren","sequence":"additional","affiliation":[{"name":"Children's Heart Center The Queen Silvia Children's Hospital Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Sunneg\u00e5rdh","sequence":"additional","affiliation":[{"name":"Children's Heart Center The Queen Silvia Children's Hospital Sahlgrenska University Hospital Gothenburg Sweden"},{"name":"Institute of Clinical Sciences Gothenburg University Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,1,6]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(91)90532-P"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezv404"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2019.01.046"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951116001487"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2010.07.087"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/08035320600649333"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(03)00516-2"},{"issue":"2","key":"e_1_2_10_9_1","first-page":"23","article-title":"Clinical manifestations of Deletion 22q11.2 syndrome (DiGeorge\/Velo\u2010Cardio\u2010Facial syndrome)","volume":"7","author":"Digilio M","year":"2005","journal-title":"Images Paediatr Cardiol"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2005.083295"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951118002482"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0146380"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezt401"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2005.10.068"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezz070"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951118001427"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002460010079"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002469900502"},{"key":"e_1_2_10_19_1","first-page":"375","article-title":"Report of the New England Regional Infant Cardiac Program, by Donald C. 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