{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T23:55:54Z","timestamp":1785542154620,"version":"3.56.0"},"reference-count":9,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2021,11,11]],"date-time":"2021-11-11T00:00:00Z","timestamp":1636588800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,12,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Intra- or extrahepatic porto-caval shunts (PCSs) can account for multiorgan dysfunction with pulmonary arterial hypertension and portosystemic encephalopathy as the most serious consequences of bypass of the hepatic circulation. The ductus venosus (DV) represents a rare foetal PCS and might be persistently patent in newborns after birth. Treatment strategies include surgical ligation and percutaneous device closure. The degree of portal vein hypoplasia limits therapy making liver transplantation the only option in some of them.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>In a newborn female patient a huge persistently patent DV, known already prenatally, resulted in severe secondary portal vein hypoplasia. She presented with hyperammonaemia, elevated liver enzymes, and pulmonary hypertension. With only diminutive portal venous branches and exceedingly high portal venous pressures during test-occlusion of the DV, shunt closure was not possible. At the age of 2 years more favourable portal venous pressures allowed transcatheter device closure with a nitinol atrial septal defect occlusion device. Pulmonary artery pressures and ammonia levels normalized after the procedure without any signs of portal hypertension.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>The case highlights the importance of meticulous imaging using balloon occlusion angiography of PCSs like the DV, to search for intrahepatic portal veins. Moreover, portal vein pressure during test-occlusion can identify patients amenable for surgical or endovascular shunt closure. Occlusion devices licensed for other indications like atrial septal defect closure can be used safely in huge PCS vessels in a one-step or staged procedure. Optimal timing of the intervention should be tailored to the patient\u2019s needs<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytab455","type":"journal-article","created":{"date-parts":[[2021,11,17]],"date-time":"2021-11-17T17:56:33Z","timestamp":1637171793000},"source":"Crossref","is-referenced-by-count":2,"title":["Percutaneous treatment of a huge patent ductus venosus and severe portal vein hypoplasia using a Figulla Flex II atrial septal defect occluder in a 2-year-old infant: a case report"],"prefix":"10.1093","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8663-7456","authenticated-orcid":false,"given":"Marek","family":"Kardos","sequence":"first","affiliation":[{"name":"Department of Functional Diagnostics, Pediatric Cardiac Center, National Institute of Cardiovascular Diseases, Pod Krasnou horkou 1, 833 48 Bratislava, Slovakia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5996-7636","authenticated-orcid":false,"given":"Erwin","family":"Kitzmueller","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, University Hospital for Children and Adolescents, Pediatric Heart Centre, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0086-5114","authenticated-orcid":false,"given":"Peter","family":"Olejnik","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Pediatric Cardiac Center, National Institute of Cardiovascular Diseases, Pod Krasnou horkou 1, 833 48 Bratislava, Slovakia"},{"name":"Department of Pediatric Cardiology, Faculty of Medicine, Comenius University, Pod Krasnou horkou 1, 833 48 Bratislava, Slovakia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3131-573X","authenticated-orcid":false,"given":"Ina","family":"Michel-Behnke","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology, University Hospital for Children and Adolescents, Pediatric Heart Centre, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,11,11]]},"reference":[{"key":"2021121919120049500_ytab455-B1","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1007\/s00431-017-3058-x","article-title":"Congenital portosystemic venous shunt","volume":"177","author":"Papamichail","year":"2018","journal-title":"Eur J Pediatr"},{"key":"2021121919120049500_ytab455-B2","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1002\/hep.30817","article-title":"Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study","volume":"71","author":"Baiges","year":"2020","journal-title":"Hepatology"},{"key":"2021121919120049500_ytab455-B3","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1016\/j.jpedsurg.2015.01.009","article-title":"The classification based on intrahepatic portal system for congenital portosystemic shunts","volume":"50","author":"Kanazawa","year":"2015","journal-title":"J Pediatr Surg"},{"key":"2021121919120049500_ytab455-B4","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.ijscr.2019.11.014","article-title":"Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated with surgical shunt ligation: a case report and literature review","volume":"66","author":"Thirapattaraphan","year":"2020","journal-title":"Int J Surg Case Rep"},{"key":"2021121919120049500_ytab455-B5","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1097\/MPG.0b013e3181d9cb92","article-title":"Complications of congenital portosystemic shunts in children; therapeutic options and outcomes","volume":"51","author":"Franchi-Abella","year":"2010","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"2021121919120049500_ytab455-B6","doi-asserted-by":"crossref","first-page":"2099","DOI":"10.1016\/j.jpedsurg.2016.09.046","article-title":"Surgical strategy according to the anatomical types of congenital portosystemic shunts in children","volume":"51","author":"Matsuura","year":"2016","journal-title":"J Pediatr Surg"},{"key":"2021121919120049500_ytab455-B7","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1016\/j.jvsv.2020.08.001","article-title":"Surgical ligation of a portosystemic shunt for the treatment of type II Abernethy malformation in 12 children","volume":"9","author":"Zhang","year":"2021","journal-title":"J Vasc Surg Venous Lymphat Disord"},{"key":"2021121919120049500_ytab455-B8","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1002\/ccd.26362","article-title":"Catheter interventional treatment of congenital portosystemic venous shunts in childhood","volume":"87","author":"Knirsch","year":"2016","journal-title":"Catheter Cardiovasc Interv"},{"key":"2021121919120049500_ytab455-B9","doi-asserted-by":"crossref","first-page":"1580","DOI":"10.1007\/s00270-013-0581-7","article-title":"Staged transcatheter treatment of portal hypoplasia and congenital portosystemic shunts in children","volume":"36","author":"Bruckheimer","year":"2013","journal-title":"Cardiovasc Interv Radiol"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ehjcr\/advance-article-pdf\/doi\/10.1093\/ehjcr\/ytab455\/41132977\/ytab455.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/5\/12\/ytab455\/41821662\/ytab455.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/5\/12\/ytab455\/41821662\/ytab455.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,19]],"date-time":"2021-12-19T19:12:19Z","timestamp":1639941139000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/doi\/10.1093\/ehjcr\/ytab455\/6425685"}},"subtitle":[],"editor":[{"given":"Mohamed","family":"Al-Hiji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Flemming","family":"Javier Olsen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Sylvia","family":"Krupickova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Daniel","family":"Tardo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]},{"given":"Ross","family":"Thomson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2021,11,11]]},"references-count":9,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2021,12,3]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytab455","relation":{},"ISSN":["2514-2119"],"issn-type":[{"value":"2514-2119","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,12,1]]},"published":{"date-parts":[[2021,11,11]]},"article-number":"ytab455"}}