Liver Involvement after a Fontan Procedure for Congenital Heart Disease
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).
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.
KeywordsFontan 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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