Bolin Elijah H, Mourani Peter M, Byrum Stephanie D, Mackintosh Samuel G, Dossey Amy M, Angtuaco Michael J, Zakaria Dala, Greiten Lawrence E, Strub Graham M, Zhang Haihong, Richter Gresham T
Department of Pediatrics, Section of Cardiology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Arkansas Children's Research Institute, Little Rock, AR, USA.
Pediatr Res. 2025 Feb;97(3):1193-1198. doi: 10.1038/s41390-024-03450-7. Epub 2024 Aug 12.
Approximately 1000 children are born every year in the United States with one effective cardiac pumping chamber, or single ventricle heart disease. One of the early causes of mortality in this population is pulmonary arteriovenous malformations (PAVMs), which allow blood to bypass gas exchange in the lungs. PAVMs most frequently occur in children after superior cavopulmonary anastomosis (SCPA), a procedure that redirects venous blood from the upper body to the lungs. Because plasma proteins are in part responsible for directing angiogenesis, we hypothesized that differential protein concentrations would be observed in superior caval blood among children after SCPA according to PAVM status. We performed quantitative plasma proteomics from 11 children with PAVMs and in seven children without PAVMs; an additional 11 children with Fontan circulation were included as a reference. Among children with SCPA, there were no significant differences in the plasma proteomes for those with and without PAVMs. When comparing children with Fontan circulation to those with SCPA and PAVMs, 18 proteins exhibited differential expression (10 downregulated and eight upregulated) in superior caval plasma. These results suggest that factors other than, or in addition to, plasma proteins may be responsible for single ventricle patients' susceptibility to PAVMs after SCPA. IMPACT: What is the key message of your article? We did not identify significant differences in plasma proteins when comparing those children with and without pulmonary arteriovenous malformations (PAVMs) after superior cavopulmonary anastomosis (SCPA). What does it add to the existing literature? The etiology of PAVMs in this population is likely due to factors other than, or in addition to, differences in plasma proteins. What is the impact? Further studies are needed to identify causes of PAVMs among children after SCPA.
在美国，每年约有1000名儿童出生时患有单心室心脏病，即只有一个有效的心脏泵血腔室。该人群早期死亡原因之一是肺动静脉畸形（PAVM），它会使血液绕过肺部的气体交换。PAVM最常发生在儿童进行上腔静脉-肺动脉吻合术（SCPA）之后，该手术可将上半身的静脉血重新导向肺部。由于血浆蛋白在一定程度上负责引导血管生成，我们推测根据PAVM状态，SCPA术后儿童的上腔静脉血中会观察到不同的蛋白浓度。我们对11名患有PAVM的儿童和7名未患PAVM的儿童进行了定量血浆蛋白质组学研究；另外11名具有Fontan循环的儿童作为对照。在进行SCPA的儿童中，患有和未患PAVM的儿童血浆蛋白质组没有显著差异。将具有Fontan循环的儿童与进行SCPA且患有PAVM的儿童进行比较时，上腔静脉血浆中有18种蛋白表现出差异表达（10种下调，8种上调）。这些结果表明，除血浆蛋白之外或之外加的其他因素可能是单心室患者SCPA术后易患PAVM的原因。影响：你的文章的关键信息是什么？我们在比较上腔静脉-肺动脉吻合术（SCPA）后患有和未患肺动静脉畸形（PAVM）的儿童时，未发现血浆蛋白有显著差异。它对现有文献有何补充？该人群中PAVM的病因可能是除血浆蛋白差异之外或之外加的其他因素。有何影响？需要进一步研究以确定SCPA术后儿童PAVM的病因。
