Pre and Post-Transplant Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure.

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-IHT. STUDY DESIGN Retrospective analysis of pre-IHT and post-IHT Fontan liver disease (FALD) status using blood tests, magnetic resonance imaging (MRI) and liver biopsy within 6 months prior to IHT and 12 months post-IHT in 9 consecutive patients with Fontan. Pre- and post-IHT 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 INR (MELD-XI), AST to Platelet Ratio Index (APRI), VAST, and cardiac catheterization data were compared. RESULTS Pre-IHT maximum MELD and MELD-XI was 15 and 16 respectively. Central venous pressures and VAST scores decreased significantly Post-IHT. In 5 paired studies, Fontan Liver MRI scores maximum was 10 Pre-IHT and decreased significantly Post-IHT. Arterially enhancing nodules on MRI persisted in 2 patients post-IHT. Pre- and Post-IHT liver biopsy scores did not differ in 4 paired biopsies. CONCLUSIONS Patients with FALD and MELD < 15, MELD-XI < 16, Fontan Liver MRI scores < 10 and VAST scores < or = 2 can have successful short term IHT outcomes. Liver MRI and VAST scores improved Post-IHT. Post-IHT liver biopsy scores did not change significantly. Pre-transplant liver biopsy demonstrating fibrosis alone should not exclude consideration of IHT. Persistence of hepatic vascular remodeling and fibrosis post-IHT suggests continued surveillance for hepatic complications Post- IHT for patients with Fontan is reasonable.