Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure

J Pediatr. 2021 Feb:229:78-85.e2. doi: 10.1016/j.jpeds.2020.09.044. Epub 2020 Sep 22.


Objective: To describe the assessment of Fontan-associated liver disease and determine the clinical and imaging measures that may identify hepatic morbidity risk in isolated heart transplantation candidates and trend those measures post-isolated heart transplantation.

Study design: Retrospective analysis of pre-isolated heart transplantation and post-isolated heart transplantation Fontan-associated liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI), and liver biopsy analysis within 6 months before isolated heart transplantation and 12 months after isolated heart transplantation in 9 consecutive patients with Fontan. Pre- and post-isolated heart transplantation standard laboratory values; varices, ascites, splenomegaly, thrombocytopenia (VAST) score; Fontan liver MRI score; liver biopsy scores; Model for End-stage Liver Disease (MELD); MELD excluding the International Normalized Ratio (MELD-XI); AST to platelet ratio index, and cardiac catheterization data were compared.

Results: Pretransplantation maximum MELD and MELD-XI was 15 and 16, respectively. Central venous pressures and VAST scores decreased significantly post-transplantation. In 5 paired studies, Fontan liver MRI score maximum was 10 pretransplantation and decreased significantly post-transplantation. Arterially enhancing nodules on MRI persisted in 2 patients post-transplantation. Pretransplantation and post-transplantation liver biopsy scores did not differ in 4 paired biopsy specimens.

Conclusions: Patients with FALD and MELD <15, MELD-XI <16, Fontan liver MRI score <10, and VAST score ≤2 can have successful short-term isolated heart transplantation outcomes. Liver MRI and VAST scores improved post-transplantation. Post-transplantation liver biopsy scores did not change significantly. Pretransplantation liver biopsy demonstrating fibrosis alone should not exclude consideration of isolated heart transplantation. The persistence of hepatic vascular remodeling and fibrosis post-isolated heart transplantation suggests that continued surveillance for hepatic complications post-transplantation for patients with Fontan is reasonable.

MeSH terms

  • Adolescent
  • Ascites / diagnostic imaging
  • Biopsy
  • Central Venous Pressure
  • Child
  • Fontan Procedure / adverse effects*
  • Heart Transplantation*
  • Humans
  • Liver / diagnostic imaging
  • Liver Cirrhosis / pathology
  • Liver Diseases / diagnosis*
  • Liver Diseases / etiology
  • Liver Function Tests
  • Magnetic Resonance Imaging
  • Patient Selection*
  • Postoperative Complications
  • Retrospective Studies
  • Splenomegaly / diagnostic imaging
  • Thrombocytopenia
  • Varicose Veins / diagnostic imaging
  • Vascular Remodeling
  • Young Adult