{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T16:05:48Z","timestamp":1793117148459,"version":"4.5.0"},"reference-count":38,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2022,4,5]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic\u2010mitral atresia, aortic atresia\u2010mitral stenosis (AA\u2010MS), and aortic\u2010mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob\u2010LV), miniaturized and slit\u2010like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA\u2010MS (35%) was associated with both mortality (all other subtypes versus AA\u2010MS: interstage\u2010I: hazard ratio [HR], 2.7;\n              <jats:italic>P<\/jats:italic>\n              =0.006; overall: HR, 2.2;\n              <jats:italic>P<\/jats:italic>\n              =0.005) and MAEs (HR, 2.4;\n              <jats:italic>P<\/jats:italic>\n              =0.0009). Glob\u2010LV (57%), noticed in all patients with AA\u2010MS, 61% of patients with aortic stenosis\u2010mitral stenosis, and 19% of patients with aortic atresia\u2010mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob\u2010LV: interstage\u2010I: HR, 4.5;\n              <jats:italic>P<\/jats:italic>\n              =0.004; overall: HR, 3.4;\n              <jats:italic>P<\/jats:italic>\n              =0.0007) and MAEs (HR, 2.7;\n              <jats:italic>P<\/jats:italic>\n              =0.0007). There was no difference in mortality and MAEs between patients with AA\u2010MS and without AA\u2010MS with Glob\u2010LV (\n              <jats:italic>P<\/jats:italic>\n              &gt;0.15). Patients with AA\u2010MS (35%) or Glob\u2010LV (38%) palliated with a Blalock\u2010Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA\u2010MS: HR, 2.6;\n              <jats:italic>P<\/jats:italic>\n              =0.04; Glob\u2010 LV: HR, 2.1;\n              <jats:italic>P<\/jats:italic>\n              =0.03).\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Glob\u2010LV and AA\u2010MS are independent morphological risk factors for adverse short\u2010 and long\u2010 term outcome, especially if a Blalock\u2010Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.121.022929","type":"journal-article","created":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T09:19:36Z","timestamp":1648545576000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden"],"prefix":"10.1161","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0879-6499","authenticated-orcid":false,"given":"Katrin","family":"Fricke","sequence":"first","affiliation":[{"name":"Cardiology Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0223-6469","authenticated-orcid":false,"given":"Mats","family":"Mellander","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Institute of Clinical SciencesSahlgrenska Academy Gothenburg Sweden"},{"name":"Children\u00b4s Heart Centre Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7464-0444","authenticated-orcid":false,"given":"Katarina","family":"Hans\u00e9us","sequence":"additional","affiliation":[{"name":"Cardiology Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5018-5435","authenticated-orcid":false,"given":"Phan\u2010Kiet","family":"Tran","sequence":"additional","affiliation":[{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"},{"name":"Cardiac Surgery Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mats","family":"Synnergren","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Institute of Clinical SciencesSahlgrenska Academy Gothenburg Sweden"},{"name":"Children\u00b4s Heart Centre Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Johansson Ramgren","sequence":"additional","affiliation":[{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"},{"name":"Cardiac Surgery Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9655-7783","authenticated-orcid":false,"given":"Annika","family":"Rydberg","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences, Pediatrics Ume\u00e5 University Ume\u00e5 Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Sunneg\u00e5rdh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Institute of Clinical SciencesSahlgrenska Academy Gothenburg Sweden"},{"name":"Children\u00b4s Heart Centre Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Magnus","family":"Dal\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery Karolinska University Hospital Stockholm Sweden"},{"name":"Department of Molecular Medicine and Surgery Karolinska Institute Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunnar","family":"Sj\u00f6berg","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institute Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7477-7283","authenticated-orcid":false,"given":"Constance G.","family":"Weismann","sequence":"additional","affiliation":[{"name":"Cardiology Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9680-4972","authenticated-orcid":false,"given":"Petru","family":"Liuba,","sequence":"additional","affiliation":[{"name":"Cardiology Pediatric Heart Centre Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Pediatrics Department of Clinical Sciences Lund University Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/pr.2016.194"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951106000291"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(94)70389-2"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.92.9.262"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1067\/mtc.2000.110490"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2008.02.026"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2009.07.063"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-014-0964-9"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2018.05.003"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0683-4"},{"key":"e_1_3_2_12_2","article-title":"Hypoplastic left heart syndrome: a new paradigm for an old disease?","volume":"6","author":"Grossfeld P","year":"2019","unstructured":"Grossfeld P, Nie S, Lin L, Wang L, Anderson RH. Hypoplastic left heart syndrome: a new paradigm for an old disease? J Cardiovasc Dev Dis. 2019;6. doi: 10.3390\/jcdd6010010","journal-title":"J Cardiovasc Dev Dis"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.117.006983"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-020-02480-2"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2019.07.090"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2008.156612"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2012.03.183"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(02)73575-7"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0912461"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.4103\/0974-2069.84653"},{"key":"e_1_3_2_21_2","first-page":"16","article-title":"Perinatal risk factors for mortality after Norwood palliation of hypoplastic left heart syndrome","volume":"25","author":"Saphir SMA","year":"2015","unstructured":"Saphir SMA, Maxedius A, Niemel\u00e4 J, Liuba P. Perinatal risk factors for mortality after Norwood palliation of hypoplastic left heart syndrome. Cardiol Young. 2015;25:16\u201317.","journal-title":"Cardiol Young"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-001-0038-7"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2007.09.007"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2015.09.056"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.66.5.1078"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(92)90101-R"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2012.03.013"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(97)01041-2"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-004-0776-4"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-011-0017-6"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2019.06.003"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2003.10.002"},{"key":"e_1_3_2_33_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.cir.0000087390.94142.1d"},{"key":"e_1_3_2_34_2","doi-asserted-by":"publisher","DOI":"10.1177\/1089253216687857"},{"key":"e_1_3_2_35_2","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezw227"},{"key":"e_1_3_2_36_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-012-0396-3"},{"key":"e_1_3_2_37_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2015.06.069"},{"key":"e_1_3_2_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2012.01.016"},{"key":"e_1_3_2_39_2","doi-asserted-by":"publisher","DOI":"10.1093\/ejcts\/ezs116"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.121.022929","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T11:27:57Z","timestamp":1715599677000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.121.022929"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,5]]},"references-count":38,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2022,4,5]]}},"alternative-id":["10.1161\/JAHA.121.022929"],"URL":"https:\/\/doi.org\/10.1161\/jaha.121.022929","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,5]]},"assertion":[{"value":"2021-06-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-22","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-03-29","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e022929"}}