Evaluation of Fontan Associated Liver Disease in Pediatric Patients Treated with Isolated Orthotopic Heart Transplantation

C. J. Rust¹, F. Shaw², C. Mao¹, M. John³, R. Romero¹, J. Rosenblum^1
1Emory University, Atlanta, Georgia ²Emory University/Children’s Healthcare of Atlanta, Atlanta, Georgia ³Children’s Healthcare of Atlanta, Atlanta, Georgia

Purpose: To evaluate the status of Fontan-associated liver disease (FALD) in patients treated with isolated orthotopic heart transplant (OHT).

Methods: This retrospective analysis examines laboratory parameters, cross-sectional imaging, liver biopsy and cardiac catheterization data in 14 patients with FALD who were treated with isolated OHT at a single pediatric institution. Previously described indices such as VAST (varices, ascites, splenomegaly, and thrombocytopenia), MELD-XI (model for end stage liver disease excluding INR) and APRI (AST to platelet ratio index) as well as cross-sectional imaging, liver biopsies and cardiac catheterization data were collected and scored. Clinical comorbidities related to FALD were also examined. Paired comparisons were performed using Wilcoxon signed-rank test.

Results: Average follow up time was 5.0 years +/- 1.6 years. Central venous pressure decreased significantly post transplantation (17 vs 9; p=0.0017). APRI (0.388 vs 0.316; p=0.0962) and VAST scores (1.2 vs 0.6; p=0.0809) decreased with the ascites sub-group (10 vs 2; p=0.0047) demonstrating statistical significance. Medical comorbidities such as protein losing enteropathy, malaise and edema all were significantly reduced following transplantation (6 vs 0; 9 vs 2; 8 vs 1; p < 0.05). BMI was significantly increased following isolated OHT (19.5 vs 23.3; p=0.0132). MELD-XI scores were not significantly different post transplantation. Weight percentiles (36.7 vs 53.9; p=0.0474) were significantly different post transplantation but height percentiles (28.6 vs 32.9; p=0.3271) were not.

Conclusion: FALD is an important comorbid condition in a growing population of patients with Fontan circulation. Timely isolated OHT improves clinical findings in FALD and may halt progression of FALD in well selected patients at five years.

Identify the source of the funding for this research project: No funding