{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,1,10]],"date-time":"2025-01-10T05:22:02Z","timestamp":1736486522726,"version":"3.32.0"},"reference-count":15,"publisher":"Cambridge University Press (CUP)","issue":"10","license":[{"start":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T00:00:00Z","timestamp":1730073600000},"content-version":"unspecified","delay-in-days":27,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951124026362_as1\"><jats:title>Introduction:<\/jats:title><jats:p>Lymphatic abnormalities have been documented on magnetic resonance lymphangiography in patients with functionally univentricular circulation.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026362_as2\"><jats:title>Objective:<\/jats:title><jats:p>The aim of this study is to evaluate associations between findings on magnetic resonance lymphangiography, cardiac catheterisation and functional phenotype in patients with Fontan-Kreutzer circulation.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026362_as3\"><jats:title>Materials and Methods:<\/jats:title><jats:p>Between January 2017 and October 2019, seven patients with Fontan-Kreutzer circulation (16.57 \u00b1 7.10 years) were enrolled following ethical committee approval and attainment of informed consent. Clinical data, consecutive magnetic resonance imaging and cardiac catheterisation findings were reviewed. Qualitative lymphatic abnormality types were defined as: 1 \u2013 Little or none; 2 \u2013 Localised to the supraclavicular region; 3 \u2013 Extending into the mediastinum; and 4 \u2013 Extending into the lung. Pulmonary hypertension was defined as a transpulmonary gradient &gt; 6 mmHg. Quantitative data were assessed using Mann\u2013Whitney <jats:italic>U<\/jats:italic> tests, and <jats:italic>p<\/jats:italic> &lt; 0.05 was considered statistically significant.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026362_as4\"><jats:title>Results:<\/jats:title><jats:p>Patients with lymphatic abnormalities categorised type 1 or 2 and transpulmonary gradient \u2264 6 mmHg had a normal functional phenotype, while those with type 3 or 4 and transpulmonary gradient &gt; 6 mmHg had a failing functional phenotype.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951124026362_as5\"><jats:title>Conclusion:<\/jats:title><jats:p>Magnetic resonance lymphangiography in patients with Fontan-Kreutzer circulation and pulmonary hypertension revealed lymphatic abnormalities types 3\u20134, indicative of a failing functional phenotype compared to patients without pulmonary hypertension. If validated in larger studies, these results suggest the potential utility of this extracardiac biomarker for improving risk stratification. Integration of lymphangiography and transpulmonary gradient with functional class, atrioventricular valve function, and ejection fraction allowed for the categorisation of three distinct functional phenotypes, which may be valuable for future analyses.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951124026362","type":"journal-article","created":{"date-parts":[[2024,10,28]],"date-time":"2024-10-28T09:47:14Z","timestamp":1730108834000},"page":"2256-2260","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":0,"title":["Magnetic resonance lymphangiography abnormalities as extracardiac biomarkers of pulmonary hypertension and functional phenotype in Fontan-Kreutzer univentricular circulation"],"prefix":"10.1017","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8939-1153","authenticated-orcid":false,"given":"E.","family":"Juaneda","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D.","family":"Catalfamo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.P.","family":"Fregapani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Guevara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1893-6806","authenticated-orcid":false,"given":"A.","family":"Peirone","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Juaneda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Kreutzer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Lucino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2024,10,28]]},"reference":[{"key":"S1047951124026362_ref8","doi-asserted-by":"publisher","DOI":"10.1177\/20458940211004777"},{"key":"S1047951124026362_ref10","first-page":"1","article-title":"MRI evaluation of lymphatic abnormalities in the neck and thorax after Fontan surgery: relationship with outcome","volume":"00","author":"Biko","year":"2019","journal-title":"Radiology"},{"key":"S1047951124026362_ref2","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000696"},{"key":"S1047951124026362_ref9","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.83445"},{"key":"S1047951124026362_ref12","doi-asserted-by":"publisher","DOI":"10.14814\/phy2.14343"},{"key":"S1047951124026362_ref15","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2021.05.021"},{"key":"S1047951124026362_ref3","doi-asserted-by":"publisher","DOI":"10.1177\/2150135117720685"},{"key":"S1047951124026362_ref14","doi-asserted-by":"publisher","DOI":"10.1016\/j.cjca.2019.09.008"},{"key":"S1047951124026362_ref1","first-page":"1","article-title":"Living to tell the tale: a first-person chronicle of the development of the Fontan-Kreutzer procedure","volume":"0","author":"Kreutzer","year":"2023","journal-title":"World J Pediatr Congenit Heart Dis"},{"key":"S1047951124026362_ref5","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.83456"},{"key":"S1047951124026362_ref13","doi-asserted-by":"publisher","DOI":"10.1016\/j.cjca.2019.08.038"},{"key":"S1047951124026362_ref4","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.13.11797"},{"key":"S1047951124026362_ref7","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.52.5.575"},{"key":"S1047951124026362_ref11","doi-asserted-by":"publisher","DOI":"10.1017\/S104795112100192X"},{"key":"S1047951124026362_ref6","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2019.06.022"}],"container-title":["Cardiology in the Young"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S1047951124026362","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,9]],"date-time":"2025-01-09T13:58:09Z","timestamp":1736431089000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S1047951124026362\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10]]},"references-count":15,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2024,10]]}},"alternative-id":["S1047951124026362"],"URL":"https:\/\/doi.org\/10.1017\/s1047951124026362","relation":{},"ISSN":["1047-9511","1467-1107"],"issn-type":[{"type":"print","value":"1047-9511"},{"type":"electronic","value":"1467-1107"}],"subject":[],"published":{"date-parts":[[2024,10]]},"assertion":[{"value":"\u00a9 The Author(s), 2024. 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