{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T21:34:58Z","timestamp":1793136898309,"version":"4.5.0"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"19","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2023,10,3]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Data are rare about the incidence of severe Fontan\u2010associated liver disease (FALD) and its association with mortality. We sought to: (1) estimate the probability of developing severe FALD in patients who undergo the Fontan procedure (Fontan patients), compared with severe liver complications in patients with a ventricular septal defect; (2) assess the severe FALD\u2010mortality association; and (3) identify risk factors for developing severe FALD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      Using the Quebec Congenital Heart Disease database, a total of 512 Fontan patients and 10\u2009232 patients with a ventricular septal defect were identified. Kaplan\u2010Meier curves demonstrated significantly higher cumulative risk of severe FALD in Fontan patients (11.95% and 52.24% at 10 and 35\u2009years, respectively), than the risk of severe liver complications in patients with a ventricular septal defect (0.50% and 2.75%, respectively). At 5\u2009years, the cumulative risk of death was 12.60% in patients with severe FALD versus 3.70% in Fontan patients without FALD (log\u2010rank\n                      <jats:italic>P<\/jats:italic>\n                      =0.0171). Cox proportional hazard models identified significant associations between the development of severe FALD and congestive heart failure and supraventricular tachycardia, with hazard ratios (HRs) of 2.36 (95% CI, 1.38\u20134.02) and 2.45 (95% CI, 1.37\u20134.39), respectively. More recent Fontan completion was related to reduced risks of severe FALD, with an HR of 0.95 (95% CI, 0.93\u20130.97) for each more recent year.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">This large\u2010scale population\u2010based study documents that severe FALD in Fontan patients was associated with a &gt;3\u2010fold increase in mortality. The risk of FALD is time\u2010dependent and can reach &gt;50% by 35\u2009years after the Fontan operation. Conditions promoting poor Fontan hemodynamics were associated with severe FALD development.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.121.024034","type":"journal-article","created":{"date-parts":[[2023,9,30]],"date-time":"2023-09-30T06:54:10Z","timestamp":1696056850000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":31,"title":["Severe Fontan\u2010Associated Liver Disease and Its Association With Mortality"],"prefix":"10.1161","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8927-4945","authenticated-orcid":false,"given":"Carlos\u2010Eduardo","family":"Guerrero\u2010Chalela","sequence":"first","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec Canada"},{"name":"Beth Raby Adult Congenital Heart Disease Clinic, Jewish General Hospital Montreal Quebec Canada"},{"name":"Fundacion Cardioinfantil\u2010Instituto de Cardiologia Bogota Colombia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Therrien","sequence":"additional","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec Canada"},{"name":"Beth Raby Adult Congenital Heart Disease Clinic, Jewish General Hospital Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoni","family":"Grossman","sequence":"additional","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6400-3775","authenticated-orcid":false,"given":"Liming","family":"Guo","sequence":"additional","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aihua","family":"Liu","sequence":"additional","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1784-8206","authenticated-orcid":false,"given":"Ariane","family":"Marelli","sequence":"additional","affiliation":[{"name":"McGill Adult Unit for Congenital Heart Disease Excellence (MAUDE Unit) McGill University Health Centre Montreal Quebec 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