{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T11:09:17Z","timestamp":1784113757236,"version":"3.55.0"},"reference-count":19,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2026,2,3]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">This study evaluated the long\u2010term outcomes of asplenia syndrome with single ventricle palliation, hypothesizing that total anomalous pulmonary vein connection (TAPVC), pulmonary atresia (PA), and greater than or equal to moderate atrioventricular valve regurgitation increase mortality risk.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p xml:lang=\"en\">This retrospective review analyzed 151 patients with asplenia syndrome who underwent single ventricle palliation between 1990 and 2024. The primary end point was mortality, with risk factors assessed by Cox regression analysis over a mean follow\u2010up of 9.3\u2009years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      The median age at initial operation was 35\u2009days, including 70 neonates (46%). Overall, 62 (41%) patients died, whereas 66 (44%) patients are alive post\u2010Fontan completion. Survival probabilities at 1 and 20\u2009years were 75.7% (95% CI, 67.9\u201381.8) and 53.7% (95% CI, 44.5\u201362.0), respectively. TAPVC (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.014), PA (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.007), and greater than or equal to moderate atrioventricular valve regurgitation (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.040) emerged as independent risk factors for mortality in the overall cohort. In the TAPVC cohort, independent risk factors for mortality included PA (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.019), infracardiac TAPVC (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.045), and neonatal TAPVC repair (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.018). When stratified by the risk factors of TAPVC, PA, or greater than or equal to moderate atrioventricular valve regurgitation, survival probabilities did not differ between patients with none or 1 of these conditions (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      =0.181) but were significantly lower in those with \u22652 risk factors (\n                      <jats:italic toggle=\"no\">P<\/jats:italic>\n                      &lt;0.001, at 15\u2009years: 0, 72.9%; 1, 59.1%; \u22652, 32.8%).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Survival after single ventricle palliation for asplenia syndrome remains suboptimal, particularly when TAPVC, PA, and greater than or equal to moderate atrioventricular valve regurgitation are present in combination rather than in isolation. Improved strategies or surgical techniques are required for this complex asplenia syndrome cohort.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.125.044756","type":"journal-article","created":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T10:12:00Z","timestamp":1769767920000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Long\u2010Term Outcomes and Risk Factors for Mortality After Single Ventricle Palliation for Asplenia Syndrome"],"prefix":"10.1161","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9285-2267","authenticated-orcid":false,"given":"Yasuyuki","family":"Kobayashi","sequence":"first","affiliation":[{"name":"Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital","place":["Okayama, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5828-8404","authenticated-orcid":false,"given":"Shunji","family":"Sano","sequence":"additional","affiliation":[{"name":"Showa Medical University Toyosu Hospital","place":["Tokyo, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-6343-0466","authenticated-orcid":false,"given":"Yuhei","family":"Tokuda","sequence":"additional","affiliation":[{"name":"Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital","place":["Okayama, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0932-467X","authenticated-orcid":false,"given":"Ayari","family":"Kimura","sequence":"additional","affiliation":[{"name":"Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital","place":["Okayama, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7588-7596","authenticated-orcid":false,"given":"Steven J.","family":"Staffa","sequence":"additional","affiliation":[{"name":"Harvard Medical School","place":["Boston, USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3610-6942","authenticated-orcid":false,"given":"David","family":"Zurakowski","sequence":"additional","affiliation":[{"name":"Harvard Medical School","place":["Boston, USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3698-4836","authenticated-orcid":false,"given":"Shingo","family":"Kasahara","sequence":"additional","affiliation":[{"name":"Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital","place":["Okayama, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7820-288X","authenticated-orcid":false,"given":"Yasuhiro","family":"Kotani","sequence":"additional","affiliation":[{"name":"Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital","place":["Okayama, Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_1_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2016.01.054"},{"key":"e_1_3_1_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2005.11.036"},{"key":"e_1_3_1_4_2","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2019-3345"},{"key":"e_1_3_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2022.05.045"},{"key":"e_1_3_1_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.xjon.2024.02.011"},{"key":"e_1_3_1_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(98)70294-6"},{"key":"e_1_3_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.xjon.2021.11.012"},{"key":"e_1_3_1_9_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2012.01.014"},{"key":"e_1_3_1_10_2","doi-asserted-by":"publisher","DOI":"10.1053\/j.optechstcvs.2022.03.003"},{"key":"e_1_3_1_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2021.01.087"},{"key":"e_1_3_1_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2023.07.003"},{"key":"e_1_3_1_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.117.006917"},{"key":"e_1_3_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2014.02.076"},{"key":"e_1_3_1_15_2","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezab141"},{"key":"e_1_3_1_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2006.05.053"},{"key":"e_1_3_1_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(03)01193-0"},{"key":"e_1_3_1_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2021.07.040"},{"key":"e_1_3_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)34743-9"},{"key":"e_1_3_1_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2023.07.040"}],"container-title":["Journal 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