Background: Failed Fontan physiology is the most common indications for isolated heart transplant (IHT) in congenital heart patients. Some patients with failed Fontan physiology have developed Fontan associated liver disease (FALD) before IHT. This retrospective study describes liver outcomes after IHT in patients with Fontan procedure.
Method: We retrospectively collected data in patients with Fontan procedure who underwent IHT between January 2000 and December 2024. We investigated liver fibrosis biomarkers such as AST-to-platelet ratio index (APRI) and Fibrosis-4 (FIB-4). Portal hypertension was reported using the VAST score, which was calculated by the sum of clinical findings (Varices, Ascites, Splenomegaly, and Thrombocytopenia [platelet < 150,000]). Liver biopsy and liver imaging findings from ultrasound, CT scan, and MRI were included.
Results: Forty-three patients included 26 children and 17 adults. The mean duration of follow up was 5.2 ± 5.4 years. There were 15/25 (60%) patients who had advanced liver fibrosis on liver biopsy (fibrosis score 3-4), 22/31 (71%) who had evidence of cirrhosis from imaging, 21/43 (49%) who had ascites, 15/43 (35%) who had splenomegaly, 7/43 (16%) who had thrombocytopenia. FIB-4 and VAST score 1 year after IHT significantly decreased compared to pre-IHT (0.57 vs. 0.63, p = 0.001, 0.4 vs. 1.2, p = 0.001, respectively) but not APRI. Eight from 22 patients (36%) still had evidence of cirrhosis from imaging and 6 from 22 patients (27%) did not show any evidence of cirrhosis from imaging after IHT. Seven of 43 patients (16%) died due to heart- or infection-related causes during the follow up period. No patients were diagnosed with hepatocellular carcinoma after IHT.
Conclusion: FIB-4 and VAST score significantly improved 1 year after IHT. We speculated that liver fibrosis and portal hypertension in FALD can be reversible after IHT. Patients with cirrhosis and significant portal hypertension can have a successful IHT without liver complications. Liver outcomes after IHT in FALD are favorable.