{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:35:46Z","timestamp":1786649746326,"version":"build-2736575974"},"reference-count":18,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2025,3,1]],"date-time":"2025-03-01T00:00:00Z","timestamp":1740787200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:sec>\n                    <jats:title>Context and Aim:<\/jats:title>\n                    <jats:p>Absent pulmonary valve syndrome\u00a0(APVS) in association with tetralogy of Fallot\u00a0(TOF) is a rare congenital heart disease characterized by the aneurysmal dilatation of pulmonary arteries and associated major airway anomalies. Postoperative outcomes have been suboptimal, especially concerning dependency on positive pressure ventilation and the need for repeated cardiac reinterventions. This study aims to describe the postoperative course of these patients and to identify the risk factors associated with poor outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and Results:<\/jats:title>\n                    <jats:p>\n                      A\u00a0retrospective review of 38\u00a0patients who underwent surgery for TOF with APVS between 2001 and 2021 at a single cardiac center was conducted. There were 16\u00a0(42%) patients in the young infants group. They required longer postoperative mechanical ventilation, 77\u00a0(interquartile ranges\u00a0[IQR] 22\u2013306) versus 17\u00a0(IQR 6\u201367) hours for the older children\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      -0.009); longer inotropic support, 127\u00a0(103\u2013285) versus 60\u00a0(20\u2013108) hours for older children\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      -0.000); longer intensive care unit\u00a0(ICU) stay, 12\u00a0(4\u201329) versus 4\u00a0(2\u20137) days\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      -0.01), and longer hospital stay 23\u00a0(14\u201364) versus 15\u00a0(11\u201320) days\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      -0.02). Seven\u00a0(43%) of the younger infants\u2019 group required readmission, whereas 4\u00a0(18%) of the older children needed to be readmitted\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0&lt;\u00a00.04). Bloodstream infections\u00a0(BSIs) occurred more frequently in the infants\u2019 group\u00a0(31%) compared to older children\u00a0(4.5%)\u00a0(\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      \u00a0&lt;\u00a00.03). The need for redo surgeries and the incidence of hospital-acquired infections other than BSIs did not show differences between the groups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Age\u00a0&lt;3\u00a0months or weight\u00a0&lt;4\u00a0kg is associated with a more difficult postoperative course, characterized by longer ventilation days, a higher inotropic score, longer ICU and hospital stays, and more readmissions.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/apc.apc_248_24","type":"journal-article","created":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T18:00:23Z","timestamp":1756490423000},"page":"119-123","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Tetralogy of Fallot with absent pulmonary valve: A single center retrospective review"],"prefix":"10.4103","volume":"18","author":[{"given":"Anis","family":"Fatima","sequence":"first","affiliation":[{"name":"Department of Cardiology, Division of Paediatric Cardiac Intensive Care, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia"},{"name":"King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdulraouf","family":"Jijeh","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Division of Paediatric Cardiac Intensive Care, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia"},{"name":"King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abdulsalam","family":"Alsayad","sequence":"additional","affiliation":[{"name":"King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia"},{"name":"King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"Department of Cardiology, Division of Paediatric Cardiology, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Obayda M.","family":"Diraneyya","sequence":"additional","affiliation":[{"name":"King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia"},{"name":"King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"Department of Cardiology, Division of Paediatric Cardiology, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ghassan","family":"Shaath","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Division of Paediatric Cardiac Intensive Care, Ministry of National Guard - Health Affairs, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Centre, Riyadh, Saudi Arabia"},{"name":"King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,8,29]]},"reference":[{"key":"R1-20260420","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/1750-1172-4-2","article-title":"Tetralogy of fallot","volume":"4","author":"Bailliard","year":"2009","journal-title":"Orphanet J Rare Dis"},{"key":"R2-20260420","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1053\/j.pcsu.2004.02.010","article-title":"Tetralogy of fallot with absent pulmonary valve syndrome","volume":"7","author":"Kirshbom","year":"2004","journal-title":"Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu"},{"key":"R3-20260420","doi-asserted-by":"crossref","first-page":"e019713","DOI":"10.1161\/JAHA.120.019713","article-title":"Contemporary outcomes in tetralogy of fallot with absent pulmonary valve after fetal diagnosis","volume":"10","author":"Chelliah","year":"2021","journal-title":"J Am Heart Assoc"},{"key":"R4-20260420","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1016\/0002-9149(82)91238-3","article-title":"Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves","volume":"50","author":"Rabinovitch","year":"1982","journal-title":"Am J Cardiol"},{"key":"R5-20260420","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1161\/01.CIR.50.1.167","article-title":"Tetralogy of fallot with absent pulmonary valve. 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