{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T03:40:21Z","timestamp":1792813221422,"version":"4.5.0"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"7","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2021,4,6]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Hypocholesterolemia is a marker of liver disease, and patients with a Fontan circulation may have hypocholesterolemia secondary to Fontan\u2010associated liver disease or inflammation. We investigated circulating lipids in adults with a Fontan circulation and assessed the associations with clinical characteristics and adverse events.<\/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              We enrolled 164 outpatients with a Fontan circulation, aged \u226518\u00a0years, in the Boston Adult Congenital Heart Disease Biobank and compared them with 81 healthy controls. The outcome was a combined outcome of nonelective cardiovascular hospitalization or death. Participants with a Fontan (median age, 30.3 [interquartile range, 22.8\u201334.3 years], 42% women) had lower total cholesterol (149.0\u00b130.1\u00a0mg\/dL versus 190.8\u00b141.4\u00a0mg\/dL,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001), low\u2010density lipoprotein cholesterol (82.5\u00b125.4\u00a0mg\/dL versus 102.0\u00b134.7\u00a0mg\/dL,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001), and high\u2010density lipoprotein cholesterol (42.8\u00b112.2\u00a0mg\/dL versus 64.1\u00b116.9\u00a0mg\/dL,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001) than controls. In those with a Fontan, high\u2010density lipoprotein cholesterol was inversely correlated with body mass index (\n              <jats:italic>r<\/jats:italic>\n              =\u22120.30,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.0001), high\u2010sensitivity C\u2010reactive protein (\n              <jats:italic>r<\/jats:italic>\n              =\u22120.27,\n              <jats:italic>P<\/jats:italic>\n              =0.0006), and alanine aminotransferase (\n              <jats:italic>r<\/jats:italic>\n              =\u22120.18,\n              <jats:italic>P<\/jats:italic>\n              =0.02) but not with other liver disease markers. Lower high\u2010density lipoprotein cholesterol was independently associated with greater hazard for the combined outcome adjusting for age, sex, body mass index, and functional class (hazard ratio [HR] per decrease of 10\u00a0mg\/dL, 1.37; 95% CI, 1.04\u20131.81 [\n              <jats:italic>P<\/jats:italic>\n              =0.03]). This relationship was attenuated when log high\u2010sensitivity C\u2010reactive protein was added to the model (HR, 1.26; 95% CI, 0.95\u20131.67 [\n              <jats:italic>P<\/jats:italic>\n              =0.10]). Total cholesterol, low\u2010density lipoprotein cholesterol, and triglycerides were not associated with the combined outcome.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">The Fontan circulation is associated with decreased cholesterol levels, and lower high\u2010density lipoprotein cholesterol is associated with adverse outcomes. This association may be driven by inflammation. Further studies are needed to understand the relationship between the severity of Fontan\u2010associated liver disease and lipid metabolism.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.120.019578","type":"journal-article","created":{"date-parts":[[2021,3,31]],"date-time":"2021-03-31T09:51:58Z","timestamp":1617184318000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":22,"title":["Fontan\u2010Associated Dyslipidemia"],"prefix":"10.1161","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6401-9980","authenticated-orcid":false,"given":"Adam M.","family":"Lubert","sequence":"first","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati 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M.","family":"Urbina","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole M.","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig","family":"Alexander","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hassan","family":"Almeneisi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fred","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Cardiology Boston Children's HospitalHarvard Medical School Boston MA"},{"name":"Department of Medicine Division of Cardiovascular Medicine Brigham and Women's Hospital Harvard Medical School Boston MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew R.","family":"Leventhal","sequence":"additional","affiliation":[{"name":"Gill Heart Institute University of Kentucky Lexington KY"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nael","family":"Aldweib","sequence":"additional","affiliation":[{"name":"Department of Cardiology Boston Children's HospitalHarvard Medical School Boston MA"},{"name":"Department of Medicine Division of Cardiovascular Medicine Brigham and Women's Hospital Harvard Medical School Boston MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Mendelson","sequence":"additional","affiliation":[{"name":"Department of Cardiology Boston Children's HospitalHarvard Medical School Boston MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6107-2281","authenticated-orcid":false,"given":"Alexander R.","family":"Opotowsky","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cincinnati Children's Hospital Heart InstituteUniversity of Cincinnati College of Medicine Cincinnati OH"},{"name":"Department of Cardiology Boston Children's HospitalHarvard Medical School Boston MA"},{"name":"Department of Medicine Division of Cardiovascular Medicine Brigham and Women's Hospital Harvard Medical School Boston 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