{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,22]],"date-time":"2026-03-22T04:41:45Z","timestamp":1774154505334,"version":"3.50.1"},"reference-count":26,"publisher":"Georg Thieme Verlag KG","issue":"S 03","license":[{"start":{"date-parts":[[2020,12,31]],"date-time":"2020-12-31T00:00:00Z","timestamp":1609372800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thorac Cardiovasc Surg"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n          Background\u2003Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications.<\/jats:p><jats:p>\n          Methods\u2003This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1\u20134) and with application of an area score (0\u201312 points).<\/jats:p><jats:p>\n          Results\u2003Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13\u201360] vs. 13 [IQR: 2\u201322] days, p\u2009=\u20090.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p\u2009=\u20090.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24\/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min\u2013max: 2\u201310] vs. 2 [min\u2013max: 0\u20138]), p\u2009=\u20090.001) and greater distribution and extension of thoracic lymphatic congestion (type 3\u20134: n\u2009=\u20095\/9 vs. n\u2009=\u20091\/33, p\u2009=\u20090.001).<\/jats:p><jats:p>\n          Conclusion\u2003Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.<\/jats:p>","DOI":"10.1055\/s-0040-1722178","type":"journal-article","created":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:59:31Z","timestamp":1609462771000},"page":"e1-e9","source":"Crossref","is-referenced-by-count":16,"special_numbering":"S","title":["Association of Lymphatic Abnormalities with Early Complications after Fontan Operation"],"prefix":"10.1055","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8455-3416","authenticated-orcid":false,"given":"Sven","family":"Dittrich","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anja","family":"Weise","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Cesnjevar","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiac Surgery, University Hospital Erlangen, Erlangen, Bavaria, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oliver","family":"Rompel","sequence":"additional","affiliation":[{"name":"Radiology Institute, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andr\u00e9","family":"R\u00fcffer","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiac Surgery, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Sch\u00f6ber","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8843-7084","authenticated-orcid":false,"given":"Julia","family":"Moosmann","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Gl\u00f6ckler","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-N\u00fcrnberg, Erlangen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2020,12,31]]},"reference":[{"issue":"06","key":"ref1","doi-asserted-by":"crossref","first-page":"e014172","DOI":"10.1161\/JAHA.119.014172","article-title":"Health-related quality of life in children, adolescents, and adults with a Fontan circulation: a meta-analysis","volume":"9","author":"K H Marshall","year":"2020","journal-title":"J Am Heart Assoc"},{"issue":"05","key":"ref2","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1017\/S1047951120000864","article-title":"Treatment strategies for protein-losing enteropathy in Fontan-palliated patients","volume":"30","author":"A Schleiger","year":"2020","journal-title":"Cardiol Young"},{"issue":"05","key":"ref3","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1016\/S0022-5223(98)70406-4","article-title":"Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group","volume":"115","author":"L Mertens","year":"1998","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"04","key":"ref4","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1017\/S1047951111000102","article-title":"Protein-losing enteropathy: integrating a new disease paradigm into recommendations for prevention and treatment","volume":"21","author":"J Meadows","year":"2011","journal-title":"Cardiol Young"},{"issue":"14","key":"ref5","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1136\/heartjnl-2015-307467","article-title":"The Fontan circulation after 45 years: update in physiology","volume":"102","author":"M Gewillig","year":"2016","journal-title":"Heart"},{"issue":"05","key":"ref6","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/j.1747-0803.2007.00116.x","article-title":"Protein-losing enteropathy after Fontan operation","volume":"2","author":"J Rychik","year":"2007","journal-title":"Congenit Heart Dis"},{"issue":"04","key":"ref7","doi-asserted-by":"crossref","first-page":"1409","DOI":"10.1016\/j.athoracsur.2012.12.042","article-title":"Analysis of the risk factors for early failure after extracardiac Fontan operation","volume":"95","author":"S Ovroutski","year":"2013","journal-title":"Ann Thorac Surg"},{"key":"ref8","first-page":"CIR0000000000000696","article-title":"Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association","volume":"\u2022\u2022\u2022","author":"J Rychik","year":"2019","journal-title":"Circulation"},{"issue":"05","key":"ref9","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1017\/S1047951120000694","article-title":"Lymphopenia in adults after the Fontan operation: prevalence and associations","volume":"30","author":"T Alsaied","year":"2020","journal-title":"Cardiol Young"},{"issue":"14","key":"ref10","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.1136\/heartjnl-2015-308823","article-title":"Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review","volume":"102","author":"F E Udink Ten Cate","year":"2016","journal-title":"Heart"},{"issue":"03","key":"ref11","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1016\/S0022-5223(96)70051-X","article-title":"Protein-losing enteropathy after the Fontan operation","volume":"112","author":"R H Feldt","year":"1996","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"01","key":"ref12","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.jacc.2014.04.025","article-title":"Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation","volume":"64","author":"A S John","year":"2014","journal-title":"J Am Coll Cardiol"},{"issue":"07","key":"ref13","doi-asserted-by":"crossref","first-page":"e015318","DOI":"10.1161\/JAHA.119.015318","article-title":"Prevalence and cause of early Fontan complications: does the lymphatic circulation play a role?","volume":"9","author":"R M Ghosh","year":"2020","journal-title":"J Am Heart Assoc"},{"issue":"04","key":"ref14","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1111\/chd.12368","article-title":"Clinical phenotypes of Fontan failure: implications for management","volume":"11","author":"W M Book","year":"2016","journal-title":"Congenit Heart Dis"},{"issue":"03","key":"ref15","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1148\/radiol.2019180877","article-title":"MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome","volume":"291","author":"D M Biko","year":"2019","journal-title":"Radiology"},{"issue":"01","key":"ref16","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1177\/2150135119890555","article-title":"Protein losing enteropathy after Fontan operation: glimpses of clarity through the lifting fog","volume":"11","author":"J Rychik","year":"2020","journal-title":"World J Pediatr Congenit Heart Surg"},{"issue":"04","key":"ref17","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1007\/s00246-007-9194-8","article-title":"First description of three patients with multifocal lymphangiomatosis and protein-losing enteropathy following palliation of complex congenital heart disease with total cavo-pulmonary connection","volume":"29","author":"M Gl\u00f6ckler","year":"2008","journal-title":"Pediatr Cardiol"},{"issue":"01","key":"ref18","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/0952-8180(92)90111-D","article-title":"Predictors of body surface area","volume":"4","author":"Y Wang","year":"1992","journal-title":"J Clin Anesth"},{"issue":"03","key":"ref19","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1016\/S0022-5223(19)39927-1","article-title":"Staged surgical management of pulmonary atresia with diminutive pulmonary arteries","volume":"73","author":"C C Gill","year":"1977","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"04","key":"ref20","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1016\/S0022-5223(19)38300-X","article-title":"A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow","volume":"88","author":"S Nakata","year":"1984","journal-title":"J Thorac Cardiovasc Surg"},{"issue":"12","key":"ref21","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1055\/s-0035-1553418","article-title":"Cardiac MRI in children and adolescents who have undergone surgical repair of right-sided congenital heart disease: automated left ventricular volumes and function analysis and effects of different manual adjustments","volume":"187","author":"O Rompel","year":"2015","journal-title":"RoFo Fortschr Geb Rontgenstr Nuklearmed"},{"issue":"04","key":"ref22","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1111\/j.1747-0803.2008.00201.x","article-title":"Lymphatic obstruction and protein-losing enteropathy in patients with congenital heart disease","volume":"3","author":"J Meadows","year":"2008","journal-title":"Congenit Heart Dis"},{"issue":"03","key":"ref23","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1161\/CIRCRESAHA.115.306544","article-title":"Lymphatic system in cardiovascular medicine","volume":"118","author":"A Aspelund","year":"2016","journal-title":"Circ Res"},{"issue":"06","key":"ref24","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1177\/2150135117732529","article-title":"Immune abnormalities in patients with single ventricle circulation precede the Fontan procedure","volume":"8","author":"L Kovacikova","year":"2017","journal-title":"World J Pediatr Congenit Heart Surg"},{"issue":"02","key":"ref25","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.jpeds.2015.04.061","article-title":"Immune abnormalities in Fontan protein-losing enteropathy: a case-control study","volume":"167","author":"H S Magdo","year":"2015","journal-title":"J Pediatr"},{"issue":"03","key":"ref26","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1111\/chd.12313","article-title":"Lymphopenia in patients with single-ventricle heart disease after the Fontan operation","volume":"11","author":"M Mattes","year":"2016","journal-title":"Congenit Heart Dis"}],"container-title":["The Thoracic and Cardiovascular Surgeon"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0040-1722178.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,2,19]],"date-time":"2021-02-19T23:37:36Z","timestamp":1613777856000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0040-1722178"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,31]]},"references-count":26,"journal-issue":{"issue":"S 03","published-online":{"date-parts":[[2021,2,19]]},"published-print":{"date-parts":[[2021,12]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0040-1722178","relation":{},"ISSN":["0171-6425","1439-1902"],"issn-type":[{"value":"0171-6425","type":"print"},{"value":"1439-1902","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,12,31]]}}}