Liver Involvement after a Fontan Procedure for Congenital Heart Disease

  • Margaret Spinosa
  • Karen L. KrokEmail author
Management of Cirrhotic Patient (A Cardenas and P Tandon, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Management of the Cirrhotic Patient


Purpose of Review

Patients with congenital heart disease are living longer into adulthood. As these patients age, we see extracardiac complications of Fontan circulation. Liver dysfunction is almost universal in patients who have had a Fontan surgery. This paper seeks to describe the current understanding of the pathophysiology, diagnosis, and management of Fontan-associated liver dysfunction (FALD).

Recent Findings

In this era of noninvasive markers for liver diseases, unfortunately, these markers have been found to be less sensitive in patients with FALD. There have been small, mostly single center, reports about the need for combined heart and liver transplant in these patients.


Clinical expertise is needed to determine optimal treatment for patients with FALD and it requires a multidisciplinary team of liver and congenital heart disease experts to manage this population.


Fontan associated liver disease Congenital heart disease 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Internal Medicine, Department of MedicinePenn State Hershey Medical CenterHersheyUSA
  2. 2.Division of Gastroenterology, Department of MedicinePenn State Milton S.Hershey Medical CenterHersheyUSA

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