Outcomes of combined liver-lung transplant in pediatric patients with cystic fibrosis: An ISHLT transplant registry study.

Background: Concomitant liver and lung transplant (ConLi-LTx) may be indicated for select pediatric patients with cystic fibrosis (CF). This study aims to analyze pre-transplant predictors and post-transplant outcomes of ConLi-LTx and lung transplant (LTx) only in pediatric patients with cystic fibrosis. Methods: The ISHLT International Thoracic Organ Transplant Registry was queried for patients <18 years old with CF who underwent ConLi-LTx and LTx-only from 1994-June 2018. Data were propensity matched to LTx-only controls and analyzed with Fisher's exact, Wilcoxon rank sum tests, Kaplan-Meier methods, and Cox proportional hazards where appropriate. Results: We identified 18 ConLi-LTx who were compared to 72 LTx-only cases. No significant differences were identified in patient demographics, resting oxygen requirement prior to transplant, or six-minute walking distance at time of listing. Interestingly, ConLi-LTx patients demonstrated significantly improved overall (p=0.0220) and BOS-free survival (p=0.0167). Conclusions: There were no differences in pre-transplant predictors of simultaneous liver and lung transplantation in pediatric patients with cystic fibrosis compared to those who received LTx-only. The noted survival benefits of ConLi-LTx remains uncertain, as patients who underwent simultaneous transplant are likely well-selected as candidates for such an extensive procedure, and we hypothesize that the liver may play a role in immunologic benefit. Further research is warranted regarding pre-transplant characteristics of pediatric cystic fibrosis patients who may be considered for concomitant liver with lung transplant.