For a study, researchers sought to describe liver transplantation (LT) outcomes and cardiac disease symptoms in a current cohort of children with Alagille Syndrome (ALGS).

In the project, the databases of the Pediatric Health Information System and the Scientific Registry of Transplant Recipients were linked uniquely. All children of ≤21 receiving their first LT were identified (2002–2018). Utilizing the diagnostic coding from the Scientific Registry of Transplant Recipients, ALGS was shown to exist. Age-matched subjects with ALGS and LT recipients with biliary atresia (BA) were matched 1:2. The patient and graft survival rates were compared between groups using the Kaplan-Meier technique and log-rank test.

A total of 312 LT receivers with BA were used as the control group, while 156 LT recipients with ALGS were found. When compared to children with BA, children with ALGS were more likely to have a congenital heart disease diagnosis (80.7% vs. 16.4%; P=0.001), with 40 (25.6%) of those children needing cardiac intervention (catheter or surgical) either before or after LT. Before LT, the creatinine, laboratory MELD, and PELD scores were higher in the patients with ALGS. Children with ALGS and BA did not vary in patient or graft survival (P=0.08 and P=0.27, respectively).

Despite greater rates of congenital heart abnormalities and cardiac procedures, higher levels of creatinine, and laboratory MELD/PELD scores at the time of transplant, the study showed that patients with ALGS and BA did not have any different survival rates for the patient or the donor.