Fontan-associated liver disease: Screening, management, and transplant considerations

Juliet Emamaullee, Ali N. Zaidi, Thomas Schiano, Jeffrey Kahn, Pamela L. Valentino, Ryan E. Hofer, Timucin Taner, Joyce W. Wald, Kim M. Olthoff, John Bucuvalas, Ryan Fischer

Research output: Contribution to journalReview articlepeer-review

7 Scopus citations


Surgical innovation and multidisciplinary management have allowed children born with univentricular physiology congenital heart disease to survive into adulthood. An estimated global population of 70 000 patients have undergone the Fontan procedure and are alive today, most of whom are <25 years of age. Several unexpected consequences of the Fontan circulation include Fontan-associated liver disease. Surveillance biopsies have demonstrated that virtually 100% of these patients develop clinically silent fibrosis by adolescence. As they mature, there are increasing reports of combined heart-liver transplantation resulting from advanced liver disease, including bridging fibrosis, cirrhosis, and hepatocellular carcinoma, in this population. In the absence of a transplantation option, these young patients face a poor quality of life and overall survival. Acknowledging that there are no consensus guidelines for diagnosing and monitoring Fontan-associated liver disease or when to consider heart transplantation versus combined heart-liver transplantation in these patients, a multidisciplinary working group reviewed the literature surrounding Fontan-associated liver disease, with a specific focus on considerations for transplantation.

Original languageEnglish (US)
Pages (from-to)591-604
Number of pages14
Issue number6
StatePublished - Aug 11 2020


  • Fontan procedure
  • heart transplantation
  • liver diseases
  • liver transplantation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


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