A small retrospective analysis by my Emory colleagues (DS Rodriguez et al [Senior author R Romero]. J Pediatr 2021; 229: 78-85. Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure) examines outcomes of 9 patients with Fontan-associated liver disease (FALD) who underwent liver transplantation.

All of these patients underwent extensive evaluations. Key findings:

• Central venous pressures and VAST scores decreased significantly post-transplantation
• Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation
• Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens
• Patients with FALD and MELD <15, MELD-XI <16 (MELD XI excludes INR), Fontan liver MRI score <10, and VAST (varices, ascites, splenomegaly, thrombocytopenia) score ≤2 can have successful short-term isolated heart transplantation outcomes

My take: This study provides reassurance that heart transplantation can proceed in patients with FALD, which is helpful as hepatic fibrosis is nearly universal in this population. After transplantation, surveillance is still needed for hepatic complications including hepatocellular carcinoma.

Related blog posts:

• Aspen Webinar (2020, part 9) FALD
• More Cases of HCC after Fontan
• Hepatocellular carcinoma after Fontan Procedure
• Does Screening for Hepatocellular Carcinoma Improve Outcomes in Patients with Cirrhosis?
• “When the Cause of Liver Disease Is the Heart”