Objective: To dynamically observe the levels and activities of von Willebrand factor (vWF) and ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) in plasma of children with congenital ventricular septal defect (VSD) during perioperative period, and explore the value of plasma vWF antigen (vWF:Ag) and ADAMTS-13 activity (ADAMTS-13: AC) in evaluating vascular endothelial injury and prognosis in children with VSD.
Methods: In this cross-sectional study, a total of 74 children with VSD who underwent surgical treatment in TEDA International Cardiovascular Hospital from September 2018 to March 2019 were enrolled in the observation group. Among them, there were 28 cases of pure VSD, 32 cases of VSD combined with pulmonary hypertension, and 14 cases of VSD combined with valvular heart disease. 31 healthy children who underwent physical examination in Tianjin Children's Hospital during the same period were collected as the control group. The biochemical indexes of the children at admission were recorded. Peripheral plasma was collected at admission, postsurgery day 0 and day 1, respectively, and the levels of vWF activity (vWF:AC), vWF:Ag, ADAMTS-13 antigen (ADAMTS-13:Ag) and ADAMTS-13:AC were detected.
Results: The level of plasma vWF:Ag and vWF:AC in the observation group before surgery were significantly lower than those in the control group (P<0.001), and increased continuously, on postsurgery day 0 and day 1 (P<0.001). The level of ADAMTS-13:Ag in the observation group before surgery was significantly higher than that in the control group (P<0.001), which decreased significantly on postsurgery day 0 (P<0.001), and increased significantly on postsurgery day 1 compared with postsurgery day 0 (P=0.033). The level of ADAMTS-13:AC in the observation group before surgery was significantly lower than that in the control group (P=0.015), which decreased significantly on postsurgery day 0 (P=0.037), and increased on postsurgery day 1, but the difference was not statistically significant (P=0.051). The changes of vWF and ADAMTS-13 in the three subgroups were basically similar to the observation group. vWF: Ag/ADAMTS-13: AC ratio on postsurgery day 0 and day 1 had high diagnostic value in vascular endothelial injury (AUC=0.80, P<0.001; AUC=0.93, P<0.001). Preoperative vWF and ADAMTS-13 levels, and related baseline indicators were not correlated with postoperative infection, bleeding, thrombosis,etc.
Conclusion: Preoperative vWF: Ag, vWF: AC and ADAMTS-13: AC levels in children with VSD are low, while the level of ADAMTS-13: Ag is high. After surgery, the levels of vWF: Ag and vWF: AC are increased and the level of ADAMTS-13: Ag is decreased. The postoperative vWF: Ag/ADAMTS-13: AC ratio shows high diagnostic value in evaluating vascular endothelial injury. There is no correlation between preoperative vWF and ADAMTS-13 levels with perioperative clinical events.
题目: 血浆vWF和ADAMTS-13在评估VSD患儿术后 血管内皮损伤及预后中的应用价值.
目的: 动态观察先天性心脏病室间隔缺损（VSD）患儿围手术期血浆中血管性血友病因子（vWF）、血管性血友病因子裂解蛋白酶（ADAMTS-13）水平和活性变化，探讨VSD患儿血浆vWF抗原（vWF:Ag）和ADAMTS-13活性（ADAMTS-13:AC）对评估血管内皮损伤及预后的应用价值.
方法: 在这项横断面研究中，收集2018年9月至2019年3月期间于泰达国际心血管病医院行手术治疗的VSD患儿74例为观察组，其中单纯VSD 28例，VSD合并肺动脉高压32例，VSD合并心脏瓣膜病14例。收集同期于天津市儿童医院体检的健康儿童31例为对照组。记录患儿入院时各项生化指标，并分别留取患儿入院时、心脏手术后当天、术后1 d的外周静脉血浆，检测vWF:Ag、vWF活性（vWF:AC）、ADAMTS-13抗原（ADAMTS-13:Ag）、ADAMTS-13:AC的水平.
结果: 观察组术前vWF:Ag、vWF:AC水平明显低于对照组（均P<0.001），术后当天、术后1 d均持续明显升高（P<0.001）。观察组术前ADAMTS-13:Ag水平明显高于对照组（P<0.001），术后当天较术前明显下降（P<0.001），术后1 d较术后当天明显上升（P=0.033）。观察组术前ADAMTS-13:AC水平明显低于对照组（P=0.015），术后当天较术前明显下降（P=0.037），术后1 d较术后当天有所上升但差异无统计学意义（P=0.051）。3个亚组术后vWF、ADAMTS-13水平与观察组整体变化趋势基本相同。术后当天及术后1 d vWF:Ag/ADAMTS-13:AC比值在血管内皮损伤中具有较高诊断价值（AUC=0.80，P<0.001；AUC=0.93，P<0.001）。术前vWF和ADAMTS-13水平及相关基线指标均与术后感染、出血、血栓形成等事件无相关性.
结论: VSD患儿术前vWF:Ag、vWF:AC和ADAMTS-13:AC水平较低，而ADAMTS-13:Ag水平较高；术后vWF:Ag、vWF:AC水平上升，ADAMTS-13:Ag水平下降。术后vWF:Ag/ADAMTS-13:AC比值在评价血管内皮损伤中有较高诊断价值；术前vWF和ADAMTS-13水平与围手术期临床事件的发生无相关性.
Keywords: ADAMTS-13; congenital heart disease; ventricular septal defect; von Willebrand factor.