Introduction: Assessment of the degree of liver disease in patients with Fontan failure remains challenging as transaminases, bilirubin and synthetic function may be normal, MRI elastography cannot distinguish between hepatic congestion and fibrosis, liver biopsy may underrepresent the degree of hepatic fibrosis and patients may have asplenia making assessment of portal hypertension difficult. Given these challenges, our indications for listing patients with Fontan failure combined heart liver transplant (cHLT) have evolved over the last 15 years. We currently list patients with Fontan failure for cHLT if they have: stage 2 or greater fibrosis on liver biopsy and evidence of portal hypertension on imaging (splenomegaly and/or intra-abdominal varices and/or ascites) or varices on endoscopy. The liver is grossly inspected intra-operatively at the time of organ acceptance: if the appearance is cirrhotic, the recipient undergoes cHLT, otherwise isolated heart transplant (HT) is performed. We aimed to correlate pre-transplant markers with the intra-operative findings of cirrhosis to help better predict which patients proceed to cHLT.
Methods: Between 2006-2021, 16 patients (median age 17 years, range 10-26) with Fontan failure and FALD were listed for cHLT and either underwent cHLT (n=12) or HT alone (n=4). Descriptive statistics, Student’s t-test and Fisher’s exact test were used to analyze the data.
Results: Patients with cirrhosis on intraoperative visual inspection who underwent cHLT had a significantly higher stage of hepatic fibrosis on pre-transplant histopathology (stage 3 vs. stage 1, p=0.04) compared to those without the intra-operative finding of cirrhosis who underwent HT only. There was no significant difference between groups for the following variables: time from Fontan in years, bilirubin, platelet count, INR off anticoagulation, ALT, or shear-wave ultrasound elastography. All patients who underwent cHLT had either:
1. Stage 2 or greater hepatic fibrosis and 2 or more signs of portal hypertension (splenomegaly, varices and/or ascites)
2. Stage 2 or greater hepatic fibrosis and polysplenia/asplenia and varices and ascites
3. No liver biopsy and 3 signs of portal hypertension (splenomegaly, varices and ascites)
No patients who underwent HT alone met any of the above criteria. No patient with stage 1 hepatic fibrosis underwent cHLT.
Conclusion: In the largest cohort of cHLT registrants and recipients, we found that the degree of hepatic fibrosis on pre-transplant histopathology combined with signs of portal hypertension predicted the gross finding of cirrhosis at the time of transplant. Better stratification and scoring tools to predict which patients with Fontan failure and FALD will benefit from cHLT are still needed. Our findings may help inform transplant decision making to better predict HL versus cHLT.