Schumacher Kurt R, Gossett Jeffrey, Guleserian Kristine, Naftel David C, Pruitt Elizabeth, Dodd Debra, Carboni Michael, Lamour Jacqueline, Pophal Stephen, Zamberlan Mary, Gajarski Robert J
Department of Pediatrics, Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan.
Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois.
J Heart Lung Transplant. 2015 Sep;34(9):1169-76. doi: 10.1016/j.healun.2015.03.022. Epub 2015 Mar 30.
Post-Fontan protein-losing enteropathy (PLE) is associated with significant morbidity and mortality. Although heart transplantation (HTx) can be curative, PLE may increase the risk of morbidity before and after HTx. This study analyzed the influence of PLE influence on waiting list and post-HTx outcomes in a pediatric cohort.
Fontan patients listed for HTx and enrolled in the Pediatric Heart Transplant Study from 1999 to 2012 were stratified by a diagnosis of PLE, and the association of PLE with waiting list and post-HTx mortality, rejection, and infection was analyzed.
Compared with non-PLE Fontan patients (n = 260), PLE patients listed for HTx (n = 96) were older (11.9 years vs 7.6 years; p = 0.003), had a larger body surface area (1.1 m(2) vs 0.9 m(2); p = 0.0001), had lower serum bilirubin (0.5 vs 0.9 mg/dl; p = 0.01), lower B-type natriuretic peptide (59 vs 227 pg/ml; p = 0.006), and were less likely to be on a ventilator (3% vs 13%; p = 0.006). PLE patients had lower waiting list mortality than non-PLE Fontan patients (p < 0.0001). There were no intergroup differences for post-HTx survival or times to the first infection or rejection. PLE was not independently associated with increased post-HTx mortality at any time point.
In this multicenter cohort, the diagnosis of PLE alone was not associated with increased waiting list mortality or post-HTx morbidity or mortality. Given the limitations of our data, this analysis suggests that PLE patients in the pediatric age group have outcomes similar to their non-PLE counterparts. Additional multicenter studies of PLE patients with targeted collection of PLE-specific information will be necessary to fully delineate the risks conferred by PLE for HTx.
Fontan术后蛋白丢失性肠病（PLE）与显著的发病率和死亡率相关。尽管心脏移植（HTx）可能治愈该病，但PLE可能增加HTx前后的发病风险。本研究分析了PLE对儿科队列中等待移植名单和HTx后结局的影响。
1999年至2012年列入HTx名单并参加儿科心脏移植研究的Fontan患者，根据PLE诊断进行分层，分析PLE与等待移植名单及HTx后死亡率、排斥反应和感染的相关性。
与非PLE的Fontan患者（n = 260）相比，列入HTx名单的PLE患者（n = 96）年龄更大（11.9岁对7.6岁；p = 0.003），体表面积更大（1.1 m²对0.9 m²；p = 0.0001），血清胆红素更低（0.5对0.9 mg/dl；p = 0.01），B型利钠肽更低（59对227 pg/ml；p = 0.006），使用呼吸机的可能性更小（3%对13%；p = 0.006）。PLE患者等待移植名单上的死亡率低于非PLE的Fontan患者（p < 0.0001）。HTx后生存率或首次感染或排斥反应的时间在组间无差异。在任何时间点，PLE与HTx后死亡率增加均无独立相关性。
在这个多中心队列中，仅PLE诊断与等待移植名单死亡率增加或HTx后发病率或死亡率增加无关。鉴于我们数据的局限性，该分析表明儿科年龄组的PLE患者结局与其非PLE对应患者相似。有必要对PLE患者进行额外的多中心研究，并针对性收集PLE特异性信息，以全面描述PLE给HTx带来的风险。
