Introduction 

Fontan-associated liver disease (FALD) is an inevitable complication of Fontan physiology. Ultrasound shear-wave elastography (SWE) is a method of characterizing liver stiffness (LS). In FALD, measurements are confounded by congestive hepatopathy. There is a need for studies to evaluate SWE longitudinally to assess hepatic fibrosis post orthotopic cardiac transplantation (OHT).

Methods 

We evaluated the effects of cardiac transplant on FALD on a cohort of patients who underwent OHT at Lurie Children’s from 2019 to 2023, describing the trajectory of liver disease. We present data on SWE, including LS measured in kilopascals (kPa), and compare findings with liver biopsy congestive hepatopathy scores.   

Results

Our cohort includes five patients who underwent OHT (Figure 1). The average age at transplant was 14.6 years. 5/5 patients had elevated kPA before OHT. Post OHT, kPa scores improved for 3/5 patients, including patient 5 who had stage three fibrosis on biopsy pre OHT. Two patients continued to have elevated kPa measurements after OHT: patient 2’s SWE was only four months after transplant, while patient 4’s elevated kPa, prompted a liver biopsy 10 months after OHT, which demonstrated stage three fibrosis.

Conclusion

While it is expected for Fontan patients to have hepatic congestion before transplant, the trajectory of liver disease post OHT is not well defined. In our cohort, LS scores improved in 3/5 patients, suggesting improvement in congestion and possible fibrosis. We hypothesize that higher kPa’s are associated with persistent, irreversible FALD post OHT.