{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,28]],"date-time":"2026-05-28T01:45:12Z","timestamp":1779932712811,"version":"3.53.1"},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,2,14]],"date-time":"2021-02-14T00:00:00Z","timestamp":1613260800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,2,14]],"date-time":"2021-02-14T00:00:00Z","timestamp":1613260800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Projekt DEAL"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arch Gynecol Obstet"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Objectives<\/jats:title>\n                <jats:p>To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum\/critical pulmonary stenosis (PAIVS\/CPS).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We evaluated 82 foetuses from 01\/08 to 10\/18 in 3 centres in intervals 1 (&lt;\u200924\u00a0weeks), 2 (24\u201330\u00a0weeks) and 3 (&gt;\u200930\u00a0weeks).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>61\/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6%\u00a0of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44\/53 (83.0%) survived. 24\/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2\u2009\u00d7\u20091.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid\/mitral valve (TV\/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Tricuspid insufficiency (TI)-Vmax\u2009&gt;\u20092.5\u00a0m\/s was present in BV outcome group in75.0% (18\/24) vs. 14.3% (2\/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax \u2009&lt;\u20092.5\u00a0m\/s, TV\/MV ratio\u2009&lt;\u2009cutoff) to a score, non-BV outcome was correctly predicted when\u2009&gt;\u20091 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4\/9 (44.4%) and 5\/14 (35.7%) in our interval 2\u2009+\u20093 with predicted non-BV outcome would have been candidates for intervention. Two (1\u2009\u00d7\u2009intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>TV\/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00404-020-05929-0","type":"journal-article","created":{"date-parts":[[2021,2,18]],"date-time":"2021-02-18T18:37:00Z","timestamp":1613673420000},"page":"81-90","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome"],"prefix":"10.1007","volume":"304","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9796-517X","authenticated-orcid":false,"given":"Aline","family":"Wolter","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natalia","family":"Markert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan Sebastian","family":"Wolter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrii","family":"Kurkevych","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Degenhardt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jochen","family":"Ritgen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R\u00fcdiger","family":"Stressig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Enzensberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ivonne","family":"Bedei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carina","family":"Vorisek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanna","family":"Schenk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Graupner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Khalil","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josef","family":"Thul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Jux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Axt-Fliedner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,2,14]]},"reference":[{"issue":"6","key":"5929_CR1","doi-asserted-by":"publisher","first-page":"562","DOI":"10.1161\/01.CIR.98.6.562","volume":"98","author":"PEF Daubeney","year":"1998","unstructured":"Daubeney PEF, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA (1998) Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. Circulation 98(6):562\u2013566","journal-title":"Circulation"},{"issue":"4","key":"5929_CR2","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.jtcvs.2005.05.044","volume":"130","author":"PEF Daubeney","year":"2005","unstructured":"Daubeney PEF, Wang D, Delany DJ, Keeton BR, Anderson RH, Slavik Z et al (2005) Pulmonary atresia with intact ventricular septum: Predictors of early and medium-term outcome in a population-based study. J Thorac Cardiovasc Surg. 130(4):1\u20139","journal-title":"J Thorac Cardiovasc Surg."},{"issue":"3","key":"5929_CR3","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1017\/S1047951104003087","volume":"14","author":"U Dyamenahalli","year":"2004","unstructured":"Dyamenahalli U, McCrindle BW, McDonald C, Trivedi KR, Smallhorn JF, Benson LN et al (2004) Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients. Cardiol Young. 14(3):299\u2013308","journal-title":"Cardiol Young."},{"issue":"12","key":"5929_CR4","doi-asserted-by":"publisher","first-page":"1111","DOI":"10.1007\/s00392-011-0355-7","volume":"100","author":"G Schwedler","year":"2011","unstructured":"Schwedler G, Lindinger A, Lange PE, Sax U, Olchvary J, Peters B et al (2011) Frequency and spectrum of congenital heart defects among live births in Germany\u202f: a study of the competence network for congenital heart defects. Clin Res Cardiol 100(12):1111\u20131117","journal-title":"Clin Res Cardiol"},{"issue":"13","key":"5929_CR5","doi-asserted-by":"publisher","first-page":"1299","DOI":"10.1016\/j.jacc.2007.08.073","volume":"51","author":"HM Gardiner","year":"2008","unstructured":"Gardiner HM, Belmar C, Tulzer G, Barlow A, Pasquini L, Carvalho JS et al (2008) Morphologic and functional predictors of eventual circulation in the fetus with pulmonary atresia or critical pulmonary stenosis with intact septum. J Am Coll Cardiol. 51(13):1299\u20131308","journal-title":"J Am Coll Cardiol."},{"issue":"2","key":"5929_CR6","doi-asserted-by":"publisher","first-page":"e415","DOI":"10.1542\/peds.2006-0428","volume":"118","author":"J Salvin","year":"2006","unstructured":"Salvin J, McElhinney D, Colan S, Gauvreau K, del Nido P, Jenkins K et al (2006) Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics 118(2):e415\u2013e420","journal-title":"Pediatrics"},{"issue":"11","key":"5929_CR7","doi-asserted-by":"publisher","first-page":"1393","DOI":"10.1016\/j.echo.2006.05.021","volume":"19","author":"R Peterson","year":"2006","unstructured":"Peterson R, Levi D, Williams R, Lai W, Sklansky M, Drant S (2006) Echocardiographic predictors of outcome in fetuses with pulmonary atresia with intact ventricular septum. J Am Soc Echocardiogr. 19(11):1393\u20131400","journal-title":"J Am Soc Echocardiogr."},{"issue":"1","key":"5929_CR8","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1002\/uog.5356","volume":"32","author":"R Iacobelli","year":"2008","unstructured":"Iacobelli R, Pasquini L, Toscano A, Raimondi F, Michielon G, Sanders SP (2008) Role of tricuspid regurgitation in fetal echocardiographic diagnosis of pulmonary atresia with intact ventricular septum. Ultrasound Obstet Gynecol 32(1):31\u201335","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"6","key":"5929_CR9","doi-asserted-by":"publisher","first-page":"661","DOI":"10.1136\/hrt.81.6.661","volume":"81","author":"YV Maeno","year":"1999","unstructured":"Maeno YV, Boutin C, Hornberger LK, Mccrindle BW, Gladman G (1999) Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart 81(6):661\u2013668","journal-title":"Heart"},{"issue":"5","key":"5929_CR10","doi-asserted-by":"publisher","first-page":"699","DOI":"10.1016\/j.amjcard.2006.09.120","volume":"99","author":"KS Roman","year":"2007","unstructured":"Roman KS, Fouron J-C, Nii M, Smallhorn JF, Chaturvedi R, Jaeggi ET (2007) Determinants of outcome in fetal pulmonary valve stenosis or atresia with intact ventricular septum. Am J Cardiol. 99(5):699\u2013703","journal-title":"Am J Cardiol."},{"issue":"3","key":"5929_CR11","doi-asserted-by":"publisher","first-page":"e510","DOI":"10.1542\/peds.2008-2014","volume":"124","author":"W Tworetzky","year":"2009","unstructured":"Tworetzky W, Doff B, Marx GR, Benson CB, Brusseau R, Morash D et al (2009) In Utero Valvuloplasty for pulmonary atresia with hypoplastic right ventricle: techniques and outcomes. Pediatrics 124(3):e510\u2013e518","journal-title":"Pediatrics"},{"issue":"5","key":"5929_CR12","doi-asserted-by":"publisher","first-page":"420","DOI":"10.1159\/000502178","volume":"47","author":"I Gottschalk","year":"2020","unstructured":"Gottschalk I, Strizek B, Menzel T, Herberg U, Breuer J, Brockmeier K et al (2020) Severe pulmonary stenosis or atresia with intact ventricular septum in the fetus the natural history. Fetal Diagn Ther. 47(5):420\u2013428","journal-title":"Fetal Diagn Ther."},{"issue":"4","key":"5929_CR13","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1046\/j.1469-0705.1996.08040241.x","volume":"8","author":"D Paladini","year":"1996","unstructured":"Paladini D, Rustico M, Todros T, Palmieri S, Gaglioti P, Benettoni A et al (1996) Conotruncal anomalies in prenatal life. Ultrasound Obstet Gynecol. 8(4):241\u2013246","journal-title":"Ultrasound Obstet Gynecol."},{"issue":"10","key":"5929_CR14","doi-asserted-by":"publisher","first-page":"1350","DOI":"10.1136\/hrt.2006.108936","volume":"94","author":"E Shinebourne","year":"2008","unstructured":"Shinebourne E, Rigby M, Carvalho J (2008) Pulmonary atresia with intact ventricular septum: from fetus to adult: congenital heart disease. Hear 94(10):1350\u20131357","journal-title":"Hear"},{"issue":"3","key":"5929_CR15","doi-asserted-by":"publisher","first-page":"348","DOI":"10.1002\/uog.12403","volume":"41","author":"JS Carvalho","year":"2013","unstructured":"Carvalho JS, Allan LD, Chaoui R, Copel JA, DeVore G, Hecher K et al (2013) ISUOG Practice guidelines (updated): sonographic screening examination of the fetal heart. Ultrasound Obstet Gynecol 41(3):348\u2013359","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"6","key":"5929_CR16","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1002\/uog.2597","volume":"26","author":"C Schneider","year":"2005","unstructured":"Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP, Daubeney PEF (2005) Development of Z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 26(6):599\u2013605","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"1","key":"5929_CR17","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1002\/uog.11196","volume":"41","author":"MD Quartermain","year":"2013","unstructured":"Quartermain MD, Glatz AC, Goldberg DJ, Cohen MS, Elias MD, Tian Z et al (2013) Pulmonary outflow tract obstruction in fetuses with complex congenital heart disease: predicting the need for neonatal intervention. Ultrasound Obstet Gynecol 41(1):47\u201353","journal-title":"Ultrasound Obstet Gynecol"},{"issue":"5","key":"5929_CR18","doi-asserted-by":"publisher","first-page":"1680","DOI":"10.1016\/j.athoracsur.2014.05.085","volume":"98","author":"AW Schneider","year":"2014","unstructured":"Schneider AW, Blom NA, Bruggemans EF, Hazekamp MG (2014) More than 25 years of experience in managing pulmonary atresia with intact ventricular septum. Ann Thorac Surg. 98(5):1680\u20131686","journal-title":"Ann Thorac Surg."},{"issue":"12","key":"5929_CR19","doi-asserted-by":"publisher","first-page":"1149","DOI":"10.1002\/pd.2386","volume":"29","author":"QH Peng","year":"2009","unstructured":"Peng QH, Zhou QC, Zeng S et al (2009) Evaluation of regional left ventricular longitudinal function in 151 normal fetuses using velocity vector imaging. Prenat Diagn 29(12):1149\u20131155","journal-title":"Prenat Diagn"},{"issue":"2","key":"5929_CR20","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1159\/000357429","volume":"35","author":"A Lowenthal","year":"2014","unstructured":"Lowenthal A, Kipps AK, Brook M (2014) Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with. Fetal Diagn Ther 35(2):101\u2013107","journal-title":"Fetal Diagn Ther"},{"issue":"4","key":"5929_CR21","doi-asserted-by":"publisher","first-page":"372","DOI":"10.1002\/pd.2698","volume":"31","author":"E G\u00f3mez-Montes","year":"2011","unstructured":"G\u00f3mez-Montes E, Herraiz I, Mendoza A, Albert L, GalindoHern\u00e1ndez-Garc\u00eda JMA (2011) Pulmonary atresia \/ critical stenosis with intact ventricular septum: prediction of outcome in the second trimester. Prenat Diagn. 31(4):372\u2013379","journal-title":"Prenat Diagn."},{"issue":"5","key":"5929_CR22","doi-asserted-by":"publisher","first-page":"1516","DOI":"10.1161\/01.CIR.86.5.1516","volume":"86","author":"TM Giglia","year":"1990","unstructured":"Giglia TM, Mandell VS, Connor ARJEM Jr, Lock JE (1990) Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 86(5):1516\u20131528","journal-title":"Circulation"},{"issue":"2","key":"5929_CR23","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1002\/uog.19047","volume":"52","author":"A Tulzer","year":"2018","unstructured":"Tulzer A, Arzt W, Gitter R, Prandstetter C, Grohmann E, Mair R et al (2018) Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis. Ultrasound Obstet Gynecol. 52(2):230\u2013237","journal-title":"Ultrasound Obstet Gynecol."},{"issue":"4","key":"5929_CR24","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1016\/j.jacc.2015.05.037","volume":"66","author":"A Moon-Grady","year":"2015","unstructured":"Moon-Grady A, Morris S, Belfort M, Chmait R, Dangel J, Devlieger R et al (2015) International fetal cardiac intervention registry: a worldwide collaborative description and preliminary outcomes. J Am Coll Cardiol 66(4):388\u2013399","journal-title":"J Am Coll Cardiol"},{"issue":"3","key":"5929_CR25","doi-asserted-by":"publisher","first-page":"373","DOI":"10.1002\/uog.15876","volume":"48","author":"HM Gardiner","year":"2016","unstructured":"Gardiner HM, Kovacevic A, Tulzer G, Sarkola T, Herberg U (2016) Natural history of 107 cases of fetal aortic stenosis from a European multicenter retrospective study. Ultrasound Obstet Gynecol 48(3):373\u2013381","journal-title":"Ultrasound Obstet Gynecol"}],"container-title":["Archives of Gynecology and Obstetrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00404-020-05929-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00404-020-05929-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00404-020-05929-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,29]],"date-time":"2021-05-29T15:05:41Z","timestamp":1622300741000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00404-020-05929-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,14]]},"references-count":25,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,7]]}},"alternative-id":["5929"],"URL":"https:\/\/doi.org\/10.1007\/s00404-020-05929-0","relation":{},"ISSN":["0932-0067","1432-0711"],"issn-type":[{"value":"0932-0067","type":"print"},{"value":"1432-0711","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,14]]},"assertion":[{"value":"28 August 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 November 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 February 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"No conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}