Fontan-associated dyslipidemia

Adam M. Lubert, Tarek Alsaied, Joseph J. Palermo, Nadeem Anwar, Elaine M. Urbina, Nicole M. Brown, Craig Alexander, Hassan Almeneisi, Fred Wu, Andrew R. Leventhal, Nael Aldweib, Michael Mendelson, Alexander R. Opotowsky

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


BACKGROUND: Hypocholesterolemia is a marker of liver disease, and patients with a Fontan circulation may have hypocho-lesterolemia secondary to Fontan-associated liver disease or inflammation. We investigated circulating lipids in adults with a Fontan circulation and assessed the associations with clinical characteristics and adverse events. METHODS AND RESULTS: We enrolled 164 outpatients with a Fontan circulation, aged ≥18 years, 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, 22.8–34.3 years], 42% women) had lower total cholesterol (149.0±30.1 mg/dL versus 190.8±41.4 mg/dL, P<0.0001), low-density lipoprotein cholesterol (82.5±25.4 mg/dL versus 102.0±34.7 mg/dL, P<0.0001), and high-density lipoprotein cholesterol (42.8±12.2 mg/ dL versus 64.1±16.9 mg/dL, P<0.0001) than controls. In those with a Fontan, high-density lipoprotein cholesterol was inversely correlated with body mass index (r=−0.30, P<0.0001), high-sensitivity C-reactive protein (r=−0.27, P=0.0006), and alanine aminotransferase (r=−0.18, P=0.02) but not with other liver disease markers. Lower high-density lipoprotein cholesterol was independently associated with greater hazard for the combined outcome adjusting for age, sex, body mass index, and func-tional class (hazard ratio [HR] per decrease of 10 mg/dL, 1.37; 95% CI, 1.04–1.81 [P=0.03]). This relationship was attenuated when log high-sensitivity C-reactive protein was added to the model (HR, 1.26; 95% CI, 0.95–1.67 [P=0.10]). Total cholesterol, low-density lipoprotein cholesterol, and triglycerides were not associated with the combined outcome. CONCLUSIONS: The Fontan circulation is associated with decreased cholesterol levels, and lower high-density 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-associated liver disease and lipid metabolism.

Original languageEnglish
Article numbere019578
JournalJournal of the American Heart Association
Issue number7
StatePublished - 2021

Bibliographical note

Publisher Copyright:
© 2021 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.


  • Adult congenital heart disease
  • Fontan
  • High-density lipoprotein cholesterol
  • Lipids
  • Single ventricle

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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