The purpose of this study was to provide an update on the outcomes of liver transplantation (LT) and the clinical symptoms of heart illness in a modern cohort of children with Alagille Syndrome (ALGS). This study made use of a novel database connection between the Scientific Registry of Transplant Recipients and the Pediatric Health Information System. For this study, researchers enumerated all youngsters younger than or around 21 years old who were receiving their initial LT (2002–2018). Scientific Registry of Transplant Recipients diagnostic coding was used to confirm the presence of ALGS. ALGS patients and LT recipients with biliary atresia (BA) were age-matched 1:2. Kaplan-Meier analysis and the log-rank test were performed to determine statistical significance between groups regarding patient and graft survival. Investigators found 156 LT recipients with ALGS and paired them with 312 LT recipients with BA as a control group. Compared to children with BA, children with ALGS were more likely to have a diagnosis of congenital heart disease (80.7% vs. 16.4%; P=0.001), and 40 (25.6%) of children with ALGS needed cardiac intervention (either a catheter or surgery) before or after LT. Before LT, the patients with ALGS had higher MELD, PELD, and laboratory MELD scores. There was no difference between children with ALGS and children with BA in how long they lived (P=0.08 and P=0.27, respectively). Even though there were more congenital heart defects and heart surgeries, higher creatinine levels, and higher MELD/PELD scores in the lab at the time of transplant, this study shows that there was no difference in the survival of either the patient or the graft between those with ALGS and those with BA.

Source – journals.lww.com/jpgn/Abstract/2022/09000/Pediatric_Liver_Transplant_Survival_in_Alagille.7.aspx