Department of Pediatrics, Division of General Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, OH.
Department of Biostatistics, University of Arkansas for Medical Sciences, Little Rock, AR.
Pediatr Crit Care Med. 2020 Sep;21(9):e776-e781. doi: 10.1097/PCC.0000000000002339.
Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot.
Retrospective cohort study.
Pediatric Health Information System.
Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion.
Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037).
The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.
照顾患有胃造口术和/或气管造口术的儿童会导致父母承受可衡量的压力。一般来说，患有 22q11.2 缺失综合征的儿童在心脏手术后更有可能需要胃造口术或气管造口术，尽管完全修复法洛四联症后的这种风险程度尚未描述。我们试图确定 22q11.2 缺失与法洛四联症修复后术后胃造口术和/或气管造口术的相关性。
回顾性队列研究。
儿科健康信息系统。
2003 年至 2016 年期间接受完全法洛四联症修复（室间隔缺损闭合和右心室流出道梗阻缓解）的儿童。如果患者患有肺动脉闭锁、其他先天性心脏缺陷和/或除 22q11.2 缺失以外的遗传诊断，则将其排除在外。
根据 22q11.2 缺失状态形成两组。结果为术后气管造口术和术后胃造口术。进行了二元分析和术后 150 天的 Kaplan-Meier 分析。有 4800 名患者，其中 317 名（7%）有 22q11.2 缺失的代码。两组间手术时年龄或性别无显著差异。22q11.2 缺失患者胃造口术（18%比 5%；p<0.001）和气管造口术（7%比 1%；p<0.001）的发生率明显更高；死亡率无差异。Kaplan-Meier 分析也显示胃造口术（p=0.024）和气管造口术（p=0.037）的发生率更高。
本研究确立了 22q11.2 缺失患者完全修复法洛四联症后术后胃造口术和气管造口术的发生率。这些数据对临床医生为患者提供术前咨询很有用。
