Marshall W. Wallace, Cameron M. Arkin, Anastasia M Kahan, Chinedu Nwaduru, Francisca van der Schyff, Jean F. Botha, Zachary J. Kastenberg
INTRODUCTION: Biliary atresia (BA) is the most common indication for pediatric liver transplantation. Pediatric end-stage liver disease (PELD) scores primarily determine deceased donor liver transplant (DDLT) waitlist priority. Due to persistent donor supply-demand mismatch, children typically have advanced disease and high PELD scores at the time of DDLT. Proactive living donor liver transplant (LDLT) may facilitate transplantation before critical illness. We hypothesized that early, low PELD, LDLT is associated with improved graft and patient survival in children with BA. METHODS: We retrospectively reviewed children undergoing liver transplantation for BA in the SRTR database, comparing DDLT to LDLT, stratified by PELD. Early transplant was defined as transplant with a PELD ≤ 12. Four groups were analyzed: low PELD LDLT, low PELD DDLT, high PELD LDLT, and high PELD DDLT. Kaplan-Meier and Cox-proportional hazards analyses evaluated patient- and graft-survival. RESULTS: 4733 children meeting inclusion criteria were included (n = 249 Low PELD LDLT, n = 1152 Low PELD DDLT, n = 708 High PELD LDLT, n = 2624 High PELD DDLT). Prior to transplant, Low PELD LDLT recipients had lower INR and bilirubin levels, and required less life support (p < 0.01). Kaplan-Meier analyses showed the highest 5-year graft and patient survival in the Low PELD LDLT (p < 0.01). Cox modeling revealed an independent association between low PELD LDLT and decreased graft loss (adjusted HR 0.45, p < 0.01) and mortality (adjusted HR 0.43, p = 0.03). CONCLUSIONS: A proactive approach to LDLT in children with BA is associated with improved long-term graft and patient survival. Low PELD LDLT should be considered to increase early transplant access for children with BA.