{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T18:30:40Z","timestamp":1773772240018,"version":"3.50.1"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,6,5]],"date-time":"2023-06-05T00:00:00Z","timestamp":1685923200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ultrasound in Obstet &amp;amp; Gyne"],"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>A favorable postnatal prognosis in cases of pulmonary atresia\/critical stenosis with intact ventricular septum (PA\/CS\u2010IVS) is generally equated with the possibility of achieving biventricular (BV) repair. Identification of fetuses that will have postnatal univentricular (UV) circulation is key for prenatal counseling, optimization of perinatal care and decision\u2010making regarding fetal therapy. We aimed to evaluate the accuracy of published models for predicting postnatal circulation in PA\/CS\u2010IVS using a large internationally derived validation cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This was a systematic review of published uni\u2010 and multiparametric models for the prediction of postnatal circulation based on echocardiographic findings at between 20 and 28\u2009weeks of gestation. Models were externally validated using data from the International Fetal Cardiac Intervention Registry. Sensitivity, specificity, predictive values, area under the receiver\u2010operating\u2010characteristics curves (AUCs) and proportion of cases with true <jats:italic>vs<\/jats:italic> predicted outcome were calculated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eleven published studies that reported prognostic parameters of postnatal circulation were identified. Models varied widely in terms of the main outcome (UV (<jats:italic>n<\/jats:italic>\u2009=\u20093), non\u2010BV (<jats:italic>n<\/jats:italic>\u2009=\u20093), BV (<jats:italic>n<\/jats:italic>\u2009=\u20093), right\u2010ventricle\u2010dependent coronary circulation (<jats:italic>n<\/jats:italic>\u2009=\u20091) or tricuspid valve size at birth (<jats:italic>n<\/jats:italic>\u2009=\u20091)) and in terms of the  included predictors (single parameters only (<jats:italic>n<\/jats:italic>\u2009=\u20096), multiparametric score (<jats:italic>n<\/jats:italic>\u2009=\u20094) or both (<jats:italic>n<\/jats:italic>\u2009=\u20091)), and were developed on small sample sizes (range, 15\u201338). Nine models were validated externally given the availability of the required parameters in the validation cohort. Tricuspid valve diameter <jats:italic>Z<\/jats:italic>\u2010score, tricuspid regurgitation, ratios between right and left cardiac structures and the presence of ventriculocoronary connections (VCC) were the most commonly evaluated parameters. Multiparametric models including up to four variables (ratios between right and left structures, right ventricular inflow duration, presence of VCC and tricuspid regurgitation) had the best performance (AUC, 0.80\u20130.89). Overall, the risk of UV outcome was underestimated and that of BV outcome was overestimated by most models.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Current prenatal models for the prediction of postnatal outcome in PA\/CS\u2010IVS are heterogeneous. Multiparametric models for predicting UV and non\u2010BV circulation perform well in identifying BV patients but have low sensitivity, underestimating the rate of fetuses that will ultimately have UV circulation. Until better discrimination can be achieved, fetal interventions may need to be limited to only those cases in which non\u2010BV postnatal circulation is certain. \u00a9 2023 The Authors. <jats:italic>Ultrasound in Obstetrics &amp; Gynecology<\/jats:italic> published by John Wiley &amp; Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.<\/jats:p><\/jats:sec>","DOI":"10.1002\/uog.26176","type":"journal-article","created":{"date-parts":[[2023,2,13]],"date-time":"2023-02-13T08:04:45Z","timestamp":1676275485000},"page":"14-22","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Prediction of postnatal circulation in pulmonary atresia\/critical stenosis with intact ventricular septum: systematic review and external validation of models"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9456-4100","authenticated-orcid":false,"given":"C.","family":"Villala\u00edn","sequence":"first","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics and Gynecology, University Hospital 12 de Octubre Complutense University  Madrid Spain"},{"name":"Research Institute Hospital 12 de Octubre (imas12)  Madrid Spain"},{"name":"Primary Care Interventions to Prevent Maternal and Child Chronic Diseases of Perinatal and Developmental Origin (RICORS network), RD21\/0012\/0024  Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A. J.","family":"Moon\u2010Grady","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Cardiology University of California San Francisco, UCSF Benioff Children's Hospital  CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Herberg","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Kinderkardiologie Universit\u00e4tsklinikum  Aachen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Strainic","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Cardiology, The Congenital Heart Collaborative, Rainbow Babies and Children's Hospital University Hospitals of Cleveland Medical Center, Case Western University  Cleveland OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7627-3094","authenticated-orcid":false,"given":"J. L.","family":"Cohen","sequence":"additional","affiliation":[{"name":"Icahn School of Medicine at Mount Sinai  New York NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Shah","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, Morgan Stanley Children's Hospital of New York Columbia University Irving Medical Center  New York NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. S.","family":"Levi","sequence":"additional","affiliation":[{"name":"Division of Cardiology, UCLA Mattel Children's Hospital University of California Los Angeles Medical School  Los Angeles CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"G\u00f3mez\u2010Montes","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics and Gynecology, University Hospital 12 de Octubre Complutense University  Madrid Spain"},{"name":"Research Institute Hospital 12 de Octubre (imas12)  Madrid Spain"},{"name":"Primary Care Interventions to Prevent Maternal and Child Chronic Diseases of Perinatal and Developmental Origin (RICORS network), RD21\/0012\/0024  Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6807-4944","authenticated-orcid":false,"given":"I.","family":"Herraiz","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics and Gynecology, University Hospital 12 de Octubre Complutense University  Madrid Spain"},{"name":"Research Institute Hospital 12 de Octubre (imas12)  Madrid Spain"},{"name":"Primary Care Interventions to Prevent Maternal and Child Chronic Diseases of Perinatal and Developmental Origin (RICORS network), RD21\/0012\/0024  Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Galindo","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics and Gynecology, University Hospital 12 de Octubre Complutense University  Madrid Spain"},{"name":"Research Institute Hospital 12 de Octubre (imas12)  Madrid Spain"},{"name":"Primary Care Interventions to Prevent Maternal and Child Chronic Diseases of Perinatal and Developmental Origin (RICORS network), RD21\/0012\/0024  Madrid Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,6,5]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/uog.63"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2014.05.085"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-0428"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1010-7940(03)00378-6"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(00)01220-0"},{"key":"e_1_2_8_7_1","first-page":"1","article-title":"Moon\u2010Grady AJ; for the IFCIR Participants. 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