Fontan-Associated Liver Disease: Screening, Management, and Transplant Considerations

Abstract

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.

Keywords: Fontan procedure; heart transplantation; liver diseases; liver transplantation.

Figure 1:

Fontan-Associated Liver Disease.

Figure 2:. Approach to surveillance of FALD.

Caveats: MR may be challenging in patients with pacemaker devices, and shearwave elastography for liver stiffness and CT abdomen/pelvis with contrast to assess for stigmata of portal hypertension can be considered in these patients. Transvenous biopsy may be safer than percutaneous approach for patients at higher risk of bleeding (blood thinners, thrombocytopenia, etc). There are no validated modalities or criteria for diagnosing HCC in the setting of FALD. Current HCC screening guidelines from the AASLD recommend AFP and US every six months in patients with cirrhosis.

Figure 3:

Transplant Considerations for patients post-Fontan.

Figure 4:. Algorithm for Heart Transplant Alone vs. Combined Heart-Liver Transplant in Patients with FALD.

Caveats: *Transvenous biopsy may be safer than percutaneous approach for patients at higher risk of bleeding (blood thinners, thrombocytopenia, etc). **Decision making for patients with histologic evidence of bridging fibrosis or cirrhosis whose liver disease remains compensated is complex and will require multidisciplinary discussion and further study. ***There may be immunologic benefit of CHLT for patients with high PRA. This has not been studied in patients post-Fontan.