Department of Pediatric Cardiology and Intensive Care Medicine, University Children's Hospital Tübingen, Tuebingen, Germany.
Department of Anesthesiology and Intensive Care Medicine, University Hospital Tübingen, Tuebingen, Germany.
J Thromb Haemost. 2018 Nov;16(11):2150-2158. doi: 10.1111/jth.14208. Epub 2018 Sep 16.
Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications. CHD-related anatomic features such as valve stenoses or patent arterial ducts can lead to enhanced shear stress in the blood stream and thus cause acquired von Willebrand syndrome (aVWS). Objective To evaluate the intraoperative incidence and impact of aVWS after cardiopulmonary bypass (CPB) in neonates and infants with complex CHD. Patients/Methods We conducted a survey of patients aged < 12 months undergoing complex cardiac surgery in our tertiary referral center. Twelve patients, whose blood samples were analyzed for aVWS before CPB and immediately after discontinuation of CPB on a routine basis, were eligible for the analysis. von Willebrand factor antigen (VWF:Ag), ristocetin cofactor activity (VWF:RCo), collagen binding activity (VWF:CB), VWF:multimers and factor VIII activity (FVIII:C) were determined. Results aVWS was diagnosed by VWF multimer analysis in 10 out of 12 patients (83%) prior to surgery and intraoperatively at the end of CPB in 8 out of 12 patients (66%). Ten patients received VWF/FVIII concentrate intraoperatively as individual treatment attempts during uncontrolled bleeding. They tolerated it well without intraoperative thrombotic events. One patient suffered a transient postoperative cerebral sinuous vein thrombosis. Conclusions aVWS is of underestimated incidence in complex CHD surgery. These data may offer a new approach to reduce the risk of severe bleedings and to achieve hemostasis during high-risk pediatric cardiac surgery by tailoring the substitution with von Willebrand factor concentrate.
新生儿先天性心脏病手术中出血并发症非常常见。我们报告了 12 例婴儿围手术期获得性血管性血友病综合征（aVWS）的发生率。12 例新生儿和婴儿中有 8 例在体外循环后术中被发现存在 aVWS。10 例患者术中接受了血管性血友病因子浓缩物，耐受性良好。
新生儿和婴儿复杂先天性心脏病（CHD）的心脏手术与术中出血并发症的高发生率相关。CHD 相关的解剖特征，如瓣膜狭窄或未闭的动脉导管，可导致血流中的剪切应力增强，从而导致获得性血管性血友病综合征（aVWS）。
评估体外循环（CPB）后复杂 CHD 新生儿和婴儿中 aVWS 的术中发生率和影响。
患者/方法：我们对在我们的三级转诊中心接受复杂心脏手术的<12 个月龄患者进行了一项调查。12 例患者的血液样本在 CPB 前和 CPB 停止后立即进行了 aVWS 分析，符合分析条件。血管性血友病因子抗原（VWF：Ag）、瑞斯托霉素辅因子活性（VWF：RCo）、胶原结合活性（VWF：CB）、VWF 多聚体和因子 VIII 活性（FVIII：C）均进行了测定。
aVWS 在术前通过 VWF 多聚体分析在 12 例患者中的 10 例（83%）中被诊断，在 12 例患者中的 8 例（66%）术中 CPB 结束时被诊断。10 例患者在术中接受了 VWF/FVIII 浓缩物作为失控性出血的个体化治疗尝试。他们耐受性良好，无术中血栓事件。1 例患者术后短暂出现脑直窦血栓形成。
在复杂 CHD 手术中，aVWS 的发生率被低估。这些数据可能为降低高危儿科心脏手术中严重出血风险和实现止血提供新方法，方法是通过调整血管性血友病因子浓缩物的替代来实现。
