A Pyogenic Liver Abscess in the Setting of Fontan-Associated Liver Disease - open access case report

Sep 28, 2026

Uploaded by ahduag

The Fontan procedure is widely used for single ventricle congenital heart disease but can lead to Fontan-associated liver disease (FALD) driven by unique Fontan hemodynamics. FALD ranges from mild hepatocellular injury to decompensated cirrhosis, progressing at different rates by patient. Because late-stage FALD carries substantial morbidity and mortality, understanding pathophysiology and complications is essential. This report describes a unique case of a pyogenic liver abscess in a patient with FALD, possibly the first such case reported.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages5

Reading Time13 min

AccessFree to download

Abstract

Introduction

Case Presentation

Review began 11/03/2025 Review ended 11/25/2025 Published 12/01/2025 © Copyright 2025 Mason et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited. DOI: 10.7759/cureus.98255 Open Access Case Report A Pyogenic Liver Abscess in the Setting of Fontan-Associated Liver Disease Thomas J. Mason 1, John A. Gambril 2 , Joshua Spegman 1, Matthew Laubham 3 1. Internal Medicine-Pediatrics, The Ohio State University, Nationwide Children 's Hospital, Columbus, USA 2. Cardiology, University of Alabama at Birmingham School of Medicine, Birmingham, USA 3. Adult Congenital Heart Disease, The Ohio State University, Nationwide Children 's Hospital, Columbus, USA Corresponding author: Thomas J. Mason, [tmac3621@gmail.com](tmac3621@gmail.com) Abstract The Fontan procedure is widely used today in the surgical management of congenital heart disease as the final step in single ventricle palliation pathways. It is associated with unique complications, most notably Fontan-associated liver disease (FALD), which is a condition arising from the unique hemodynamics ofthe Fontan circulation that occurs universally after the procedure. Though varying in severity from mild hepatocellular liver injury to frank decompensated cirrhosis, FALD progresses at different rates depending on the patient. Given the significant morbidity and mortality associated with late-stage FALD, a better understanding of the pathophysiology and complications is needed. Here we describe a unique case of apyogenic liver abscess in a patient with FALD, possibly the first reported in the literature. Categories: Cardiology, Anatomy, Gastroenterology Keywords: adult congenital heart disease (achd), congenital heart diseases (chds), congestive hepatopathy, fontanassociated liver disease, fontan circulation, fontan physiology, pyogenic liver abscess (pla) Introduction In 1971, Francis Fontan pioneered the first total subpulmonic ventricular bypass procedure to improve cyanosis in a patient with tricuspid atresia. After various modifications, the eponymous Fontan procedure has become the standard of care in the management of single ventricle congenital heart disease as the final step in surgical palliation [1] . Briefly, the Fontan procedure creates a pathway for lower extremity venous blood to bypass the heart and flow directly into the pulmonary arteries. When coupled with a Glenn shunt, which allows the flow of upper extremity venous blood into the pulmonary arteries, the Fontan procedure allows complete passive cavopulmonary blood flow. This serves to improve cyanosis and systemic ventricular volume load in patients with congenital single ventricle heart disease of many forms. It is estimated that 50,000-70,000 people worldwide are living with Fontan circulation, with 40-55% of these being adults [2,3] . Surgical and transcatheter advancements, as well as improved medical management of Fontan-associated comorbidities, have greatly improved survival, with a contemporary estimated 30-year survival rate of 85%[2] . With improved survival, the frequency of newer complications has increased, like Fontan-associated liver disease (FALD) . FALD encompasses the spectrum of functional and structural alterations of the liver, including synthetic dysfunction and fibrosis, and if progressive, frank cirrhosis, portal hypertension, and hepatocellular carcinoma (HCC) [4] . The pathophysiology of FALD includes elevated systemic venous pressure leading to inefficient blood drainage from the liver, creating chronic passive congestion and noninflammatory fibrogenesis. Clinical presentation of FALD is variable, but can be characterized by elevated liver enzymes, abnormal hepatocyte synthetic function, esophageal or gastric varices, splenomegaly, ascites, and encephalopathy, depe

FAQs

What is Fontan-associated liver disease (FALD) in patients after the Fontan procedure?

FALD is a liver condition caused by the unique hemodynamics of Fontan circulation. It can progress from functional and structural liver alterations to cirrhosis, portal hypertension, and hepatocellular carcinoma.

What makes this case report notable?

It describes a pyogenic liver abscess occurring in the setting of FALD, described as possibly the first reported case in the literature.

How did the patient present and what were the key early findings?

The patient had about one week of fevers and malaise, then acute emesis and diarrhea. He was hypotensive and in shock with high lactate, multiorgan dysfunction, elevated inflammatory markers, and blood culture growth of Streptococcus intermedius.

Sep 28, 2026

Uploaded by ahduag