Keck School of Medicine , University of Southern California , Los Angeles , California , USA.
Division of Hepatobiliary and Abdominal Transplant Surgery, Department of Surgery , University of Southern California , Los Angeles , California , USA.
Liver Transpl. 2023 Feb 1;29(2):134-144. doi: 10.1002/lt.26548. Epub 2023 Jan 17.
Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End-Stage Liver Disease (MELD)/Pediatric End-Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States 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. A retrospective cohort of patients aged younger than 18 years with a MELD/PELD exception request who underwent LT between 2007 and 2018 were identified in the Scientific Registry of Transplant Recipients. Patients were stratified by waitlist partial pressure of arterial oxygen (PaO 2 ) to assess risk factors for waitlist mortality and post-LT survival. Among 3082 pediatric LT recipients included in the study, 124 patients (4%) received MELD/PELD exception points for HPS. Patients with HPS were a median age of 9 years (interquartile range: 6, 12 years), 54.8% were girls, and 54% were White. Most patients (87.9%) were listed with laboratory MELD/PELD scores <15. Waitlist mortality for patients with HPS exception points was rare and not different from patients without HPS. When stratified by pre-LT PaO 2 , hypoxemia severity was not associated with differences in 1-, 3-, or 5-year survival rates after LT ( p = 0.13). However, patients with HPS showed a slightly lower survival rate at 5 years compared with patients without HPS (88.7% vs. 93.4%; p = 0.04). MELD/PELD exceptions for children with HPS mitigated waitlist mortality, and recipients with HPS experienced excellent 5-year survival after LT, although slightly lower than in patients without HPS. Unlike adults with HPS, the 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 the risk factors predicting negative survival outcomes in children with HPS are warranted.
肝肺综合征（HPS）与肝移植（LT）候选者的等待名单死亡率增加有关。根据为成年人制定的标准，美国的 HPS 患儿在等待名单上获得终末期肝病模型（MELD）/小儿终末期肝病（PELD）的例外积分，以优先排序。在这项研究中，检查了这种 MELD/PELD 例外政策对 LT 后儿童生存的影响。在 Scientific Registry of Transplant Recipients 中，确定了 2007 年至 2018 年间接受 LT 的年龄小于 18 岁且有 MELD/PELD 例外请求的患者的回顾性队列。根据等待名单动脉氧分压（PaO 2 ）对患者进行分层，以评估等待名单死亡率和 LT 后生存率的危险因素。在包括的 3082 名儿科 LT 受者中，有 124 名患者（4%）因 HPS 获得 MELD/PELD 例外积分。HPS 患者的中位年龄为 9 岁（四分位距：6，12 岁），54.8%为女孩，54%为白人。大多数患者（87.9%）的实验室 MELD/PELD 评分<15 被列入名单。HPS 例外点患者的等待名单死亡率罕见，与无 HPS 患者无差异。当按 LT 前 PaO 2 分层时，低氧血症严重程度与 LT 后 1、3 或 5 年生存率无差异（p=0.13）。然而，与无 HPS 患者相比，HPS 患者的 5 年生存率略低（88.7% vs. 93.4%；p=0.04）。MELD/PELD 对 HPS 患儿的例外减轻了等待名单死亡率，接受 HPS 的患儿 LT 后 5 年生存率极佳，尽管略低于无 HPS 患儿。与 HPS 成人不同，儿童 LT 前低氧血症的严重程度并不影响 LT 后生存率。这些数据表明，给予 MELD/PELD 例外积分的成人标准可能无法恰当地反映儿科患者的 HPS 严重程度。需要进一步的前瞻性多中心研究来检查预测 HPS 患儿生存结果不良的危险因素。
