Abstract：
Previous research suggests that individuals with 22q11.2 deletion syndrome (DS) have an increased risk of bleeding following cardiac surgery. However, current guidelines for management of patients with 22q11.2DS do not provide specific recommendations for perioperative management. This study sought to identify specific risk factors for bleeding in this patient population. Examine the factors determining bleeding and transfusion requirements in patients with 22q11.2DS undergoing cardiac surgery. This was a single center review of patients who underwent cardiac surgery at the Children\'s Hospital of Philadelphia from 2000 to 2016. Data was extracted from the medical record. Frequency of bleeding events, laboratory values, and transfusion requirements were compared. We included 226 patients with 22q11.2DS and 506 controls. Bleeding events were identified in 13 patients with 22q11.2DS (5.8%) and 27 controls (5.3%). Platelet counts were lower among patients with 22q11.2DS than in control patients, but not statistically different comparing bleeding to not bleeding. Patients with 22q11.2DS received more transfusions (regardless of bleeding status). However, multivariate analysis showed only procedure type was associated with increased risk of bleeding (p = .012). The overall risk of bleeding when undergoing cardiac surgery is not different in patients with 22q11.2DS compared to non-deleted patients. Though platelet counts were lower in patients with 22q11.2DS, only procedure type was significantly associated with an increased risk of bleeding.
摘要：
先前的研究表明，患有22q11.2缺失综合征（DS）的个体在心脏手术后出血的风险增加。然而,目前22q11.2DS患者的管理指南未提供围手术期管理的具体建议.这项研究旨在确定该患者人群中出血的特定风险因素。检查决定22q11.2DS患者接受心脏手术的出血和输血需求的因素。这是2000年至2016年在费城儿童医院接受心脏手术的患者的单中心审查。数据是从医疗记录中提取的。出血事件的频率,实验室值，和输血需求进行了比较。我们纳入226名22q11.2DS患者和506名对照。在13例22q11.2DS患者（5.8％）和27例对照（5.3％）中发现了出血事件。22q11.2DS患者的血小板计数低于对照组患者，但出血和未出血的比较没有统计学差异。22q11.2DS患者接受更多输血（无论出血状态如何）。然而,多变量分析显示，只有手术类型与出血风险增加相关（p=0.012）。与未删除的患者相比，22q11.2DS患者接受心脏手术时的总体出血风险没有差异。尽管22q11.2DS患者的血小板计数较低，只有手术类型与出血风险增加显著相关.
