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 fibr...

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Main Authors: Renita Wilson, BS, J. Asher Jenkins, MD, Juan Maria Farina, MD, Blake Langlais, MS, Bashar Aqel, MD, Ashraf Omar, MD, Jonathan D’Cunha, MD PhD, Pedro Reck dos Santos, MD PhD
Format: Article
Language:English
Published: Elsevier 2025-02-01
Series:JHLT Open
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Online Access:http://www.sciencedirect.com/science/article/pii/S2950133425000072
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Summary: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.
ISSN:2950-1334