*Purpose: Fontan-associated liver disease (FALD) is a well-described complication of Fontan physiology. More patients with failing Fontan are being evaluated for heart transplant and evidence-based guidelines on who benefits from combined heart-liver transplantation(CHLT) vs. heart alone are lacking. Liver biopsy is a well-established modality for liver disease staging among patients with other causes of cirrhosis but may be not reliable in FALD patients due to non-uniform fibrosis distribution. We compare peritransplant clinical and biopsy characteristics of FALD vs non-FALD patients who underwent a CHLT at our institution to determine reliability of pre-transplant evaluation in relation to explant pathology.

*Methods: 15 consecutive patients undergoing CHLT at our center were reviewed (January 1 2017- November 1 2021). Pre transplant clinical, radiographic, pathology data were collected and correlated with explant pathology. Liver pathology slides were reviewed by two pathologists, and scored both according to Metavir system and congestive hepatic fibrosis score (CHFS).

*Results: We identified 10 patients with FALD (median age 36, range 26-51), and 5 with non-FALD (median age 50; range 37-64): congestive hepatopathy(n=2), HCV(n=1), HCV/EtOH(n=1), hemochromatosis(n=1). A total of 4 patients died, 3 in FALD and 1 in non-FALD groups. Ten patients had cirrhosis on explant, 5 had F3 and 6 had findings of both F3 and F4 stages throughout explant liver. Seven patient (5 FALD and 2 non-FALD) had pretransplant liver biopsy slides available for review. When fibrosis was scored both according to the Metavir and CHFS system, a 1:1 correlation was observed. Pretransplant liver biopsy underestimated explant pathology (by 1 stage) in 5 out of 7 patients.

*Conclusions: In our series of CHLT recipients, Metavir and CHFS scoring system staged liver fibrosis similarly; pretransplant liver biopsy in our series tends to underestimate explant pathology regardless of etiology of liver disease. Traditional liver disease staging tools may not be reliable in this patient population.

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