{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T17:54:26Z","timestamp":1790445266542,"version":"4.1.0"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2022,1,18]],"date-time":"2022-01-18T00:00:00Z","timestamp":1642464000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,1,18]],"date-time":"2022-01-18T00:00:00Z","timestamp":1642464000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum Bonn"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Arch Gynecol Obstet"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>The purpose of this study was to explore the outcome in fetuses with prenatal diagnosis of absent pulmonary valve syndrome (APVS) on ultrasound imaging.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>A manual web scraping technique was utilized, where MEDLINE and EMBASE were searched along the combination with other relevant medical subject headings such as \u201cabsent pulmonary valve syndrome\u201d, \u201cprenatal APVS\u201d and \u201cAPVS\/outcome\u201d. The observed outcomes encompassed the rate of chromosomal abnormalities, associations and malformations linked to APVS and fetuses with APVS. A quality assessment of the included studies was also performed. We used meta-analyses of proportions to combine data and fixed or random-effects models according to the heterogeneity of the results.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Seven studies including 199 fetuses with APVS were included in the analysis. The median gestational age at referral to the tertiary center was 24.8\u00a0weeks. An association to tetralogy of Fallot (TOF) could be seen in 84.4% of all cases. In total 140 out of 199 cases underwent invasive testing, with a total number of 55 abnormal karyotypes [39.3% (95% CI 31.1\u201347.9%)]. 35.2% of the patients opted for termination of pregnancy (95% CI 28.5\u201342.3%).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>The analysis underlines the distribution of fetuses with APVS, with 84.4% of cases presenting with TOF\/APVS and only 12.6% having APVS\/intact ventricular septum (IVS). Larger and more prospective study analyses is now needed, especially focusing on long-term follow-up periods of fetuses and children with APVS. Particularly as the postnatal course shows great variety depending on prenatal diagnosis.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00404-022-06397-4","type":"journal-article","created":{"date-parts":[[2022,1,18]],"date-time":"2022-01-18T17:07:49Z","timestamp":1642525669000},"page":"1449-1454","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Management and outcome of prenatal absent pulmonary valve syndrome"],"prefix":"10.1007","volume":"306","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9135-4338","authenticated-orcid":false,"given":"Florian","family":"Recker","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eva C.","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Strizek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annegret","family":"Geipel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Berg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"Gembruch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,1,18]]},"reference":[{"key":"6397_CR1","doi-asserted-by":"publisher","first-page":"266","DOI":"10.1161\/01.CIR.26.2.266","volume":"26","author":"RA Miller","year":"1962","unstructured":"Miller RA, Lev M, Paul MH (1962) Congenital absence of the pulmonary valve. The clinical syndrome of tetralogy of Fallot with pulmonary regurgitation. Circulation 26:266\u2013278","journal-title":"Circulation"},{"key":"6397_CR2","doi-asserted-by":"publisher","first-page":"1353","DOI":"10.1007\/s10554-015-0693-z","volume":"31","author":"W Wu","year":"2015","unstructured":"Wu W, Pang K, Lin Q et al (2015) Echocardiography in the diagnosis of patients with absent pulmonary valve syndrome: a review study of 12 years. Int J Cardiovasc Imaging 31:1353\u20131359","journal-title":"Int J Cardiovasc Imaging"},{"key":"6397_CR3","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1053\/j.semtcvs.2019.05.022","volume":"31","author":"DM Dorobantu","year":"2019","unstructured":"Dorobantu DM, Stoicescu C, Tulloh RM, Stoica SC (2019) Surgical repair of tetralogy of fallot with absent pulmonary valve: favorable long-term results. Semin Thorac Cardiovasc Surg 31:847\u2013849","journal-title":"Semin Thorac Cardiovasc Surg"},{"key":"6397_CR4","doi-asserted-by":"publisher","first-page":"812","DOI":"10.1002\/pd.5094","volume":"37","author":"R Axt-Fliedner","year":"2017","unstructured":"Axt-Fliedner R, Kurkevych A, Slodki M et al (2017) Absent pulmonary valve syndrome\u2014diagnosis, associations, and outcome in 71 prenatally diagnosed cases: APVS\u2014diagnosis, associations and outcome. Prenat Diagn 37:812\u2013819","journal-title":"Prenat Diagn"},{"key":"6397_CR5","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1002\/uog.15977","volume":"49","author":"I Gottschalk","year":"2017","unstructured":"Gottschalk I, Jehle C, Herberg U et al (2017) Prenatal diagnosis of absent pulmonary valve syndrome from first trimester onwards: novel insights into pathophysiology, associated conditions and outcome: absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 49:637\u2013642","journal-title":"Ultrasound Obstet Gynecol"},{"key":"6397_CR6","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.athoracsur.2014.03.002","volume":"98","author":"A Szwast","year":"2014","unstructured":"Szwast A, Tian Z, McCann M et al (2014) Anatomic variability and outcome in prenatally diagnosed absent pulmonary valve syndrome. Ann Thorac Surg 98:152\u2013158","journal-title":"Ann Thorac Surg"},{"key":"6397_CR7","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1002\/uog.11193","volume":"41","author":"D Wertaschnigg","year":"2013","unstructured":"Wertaschnigg D, Jaeggi M, Chitayat D et al (2013) Prenatal diagnosis and outcome of absent pulmonary valve syndrome: contemporary single-center experience and review of the literature: absent pulmonary valve. Ultrasound Obstet Gynecol 41:162\u2013167","journal-title":"Ultrasound Obstet Gynecol"},{"key":"6397_CR8","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1002\/uog.2807","volume":"28","author":"A Galindo","year":"2006","unstructured":"Galindo A, Guti\u00e9rrez-Larraya F, Mart\u00ednez JM et al (2006) Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol 28:32\u201339","journal-title":"Ultrasound Obstet Gynecol"},{"key":"6397_CR9","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1002\/uog.1729","volume":"24","author":"P Volpe","year":"2004","unstructured":"Volpe P, Paladini D, Marasini M et al (2004) Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 24:623\u2013628","journal-title":"Ultrasound Obstet Gynecol"},{"key":"6397_CR10","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1016\/S0002-9149(02)03238-1","volume":"91","author":"RS Razavi","year":"2003","unstructured":"Razavi RS, Sharland GK, Simpson JM (2003) Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome. Am J Cardiol 91:429\u2013432","journal-title":"Am J Cardiol"},{"key":"6397_CR11","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1046\/j.1469-0705.2001.00242.x","volume":"17","author":"R Becker","year":"2001","unstructured":"Becker R, Schmitz L, Guschmann M, Wegner RD, Stiemer B, Entezami M (2001) Prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature. Ultrasound Obstet Gynecol 17:263\u2013267","journal-title":"Ultrasound Obstet Gynecol"},{"key":"6397_CR12","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1046\/j.1540-8175.2002.00489.x","volume":"19","author":"SB Yeager","year":"2002","unstructured":"Yeager SB, Van Der Velde ME, Waters BL, Sanders SP (2002) Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome. Echocardiography 19:489\u2013493","journal-title":"Echocardiography"},{"key":"6397_CR13","doi-asserted-by":"publisher","first-page":"1280","DOI":"10.1016\/S0002-9149(02)02326-3","volume":"89","author":"AJ Moon-Grady","year":"2002","unstructured":"Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH (2002) Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol 89:1280\u20131285","journal-title":"Am J Cardiol"},{"key":"6397_CR14","doi-asserted-by":"publisher","first-page":"682","DOI":"10.1016\/j.ejcts.2006.01.050","volume":"29","author":"MA N\u00f8rgaard","year":"2006","unstructured":"N\u00f8rgaard MA, Alphonso N, Newcomb AE, Brizard CP, Cochrane AD (2006) Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardiothorac Surg 29:682\u2013687","journal-title":"Eur J Cardiothorac Surg"}],"container-title":["Archives of Gynecology and Obstetrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00404-022-06397-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00404-022-06397-4\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00404-022-06397-4.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,9,28]],"date-time":"2022-09-28T04:04:58Z","timestamp":1664337898000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00404-022-06397-4"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,18]]},"references-count":14,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["6397"],"URL":"https:\/\/doi.org\/10.1007\/s00404-022-06397-4","relation":{},"ISSN":["1432-0711"],"issn-type":[{"value":"1432-0711","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,1,18]]},"assertion":[{"value":"26 May 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 January 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 January 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no conflicts of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Not applicable.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}},{"value":"All authors gave consent for publication.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication."}}]}}