//pubmed.ncbi.nlm.nih.gov/35876731/ liver transplant

Liver Transpl. 2022 Jul 25.
doi: 10.1002/lt.26548. Online ahead of print.

MELD/PELD Exception Policy and Outcomes in Pediatric Patients with Hepatopulmonary Syndrome Requiring Liver Transplantationhttps:

Muhammad H Raza # 1, Yong Kwon # 1 2 3, Pierre Kobierski 1, Asish C Misra 1 2 3, Angelina Lim 1, Cameron Goldbeck 2, Kambiz Etesami 1 2 3, Rohit Kohli 1 3 4, Juliet Emamaullee 1 2 3

PMID: 35876731

DOI: 10.1002/lt.26548
Abstract

Background: Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplant (LT) candidates. Children with HPS are granted MELD/PELD exception points for waitlist prioritization in the U.S. based on criterion developed for adults. In this study, the impact of this MELD/PELD exception policy on post-LT survival in children was examined.

Methods: A retrospective cohort of patients <18 years with a MELD/PELD exception request who underwent LT between 2007-2018 were identified in the SRTR. Patients were stratified by waitlist PaO2 to assess risk factors for waitlist mortality and post-LT survival.

Results: Among 3,082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were median 9 [6, 12] years old, 54.8% female, and 54% white. Most patients (87.9%) were listed with a lab MELD/PELD <15. Waitlist mortality for patients with HPS exception points was rare and not different from non-HPS patients. When stratified by pre-LT PaO2 , hypoxemia severity was not associated with differences in 1, 3, or 5-year survival post-LT (p=0.13). However, HPS patients showed a slightly lower survival at 5-years compared to non-HPS patients (88.7% vs 93.4%; p=0.04).

Conclusions: MELD/PELD exception for children with HPS mitigated waitlist mortality, and recipients with HPS experience excellent 5-year survival post-LT, though slightly lower than in patients without HPS. Unlike adults with HPS, severity of pre-LT hypoxemia in children does not impact post-LT survival. These data suggest that adult criteria for granting MELD/PELD exception points may not appropriately capture HPS severity in pediatric patients. Further prospective multicenter studies to examine risk factors predicting negative survival outcomes in children with HPS are warranted.