Department of Anesthesiology, Critical Care and Pain Medicine, Harvard Medical School and Boston Children's Hospital, Boston, Massachusetts.
Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Semin Thorac Cardiovasc Surg. 2020;32(4):947-957. doi: 10.1053/j.semtcvs.2020.06.037. Epub 2020 Jul 2.
Children with Down syndrome (DS) have lower mortality compared to nonsyndromic (NS) children after atrioventricular septal defect (AVSD) repair. Limited data exist regarding hospital mortality and utilization after other congenital heart disease (CHD) operations in DS. We compared hospital mortality and utilization after CHD operations in both populations and hypothesized that the survival benefit in children with DS is not consistent across CHD lesions. The Texas Inpatient Public Use Datafile was queried for all patients <18 years old undergoing operations for CHD between 1999 and 2016. Hospital mortality, length-of-stay and charges were compared between DS and NS groups, stratified by CHD operation using mixed-effects multivariable analyses and propensity score matching analyses adjusting for prematurity, low birth weight, age, and sex. Over the 18-year period, 2841 cases with DS underwent CHD operations compared to 25,063 NS cases. The most common types of interventions performed in DS were AVSD repair, isolated ventricular septal defect (VSD) repair and tetralogy of Fallot (TOF) repair. By multivariable analyses, DS was associated with lower mortality after isolated AVSD repair (RR 0.40 [IQR 0.20-0.79]), and higher hospital mortality after bidirectional Glenn anastomosis (BDG) (RR 5.17 [IQR 2.10-12.77]) and TOF/pulmonary atresia repair (RR 9.71 [IQR 2.16-43.68]) compared to NS children. Similar results were noted using propensity score matching. Children with DS had lower mortality after AVSD repair than NS children, but higher mortality after operations for BDG and TOF/pulmonary atresia. Further study is needed to determine if the presence of pulmonary hypertension in DS modifies the association between DS and mortality depending on cardiac lesion.
唐氏综合征（DS）患儿在接受房室间隔缺损（AVSD）修复术后的死亡率低于非唐氏综合征（NS）患儿。关于 DS 患儿在接受其他先天性心脏病（CHD）手术后的院内死亡率和利用情况的数据有限。我们比较了这两种人群在 CHD 手术后的院内死亡率和利用情况，并假设 DS 患儿的生存获益在不同的 CHD 病变中并不一致。通过混合效应多变量分析和倾向评分匹配分析，调整了早产、低出生体重、年龄和性别等因素，从 1999 年至 2016 年，对德克萨斯州住院患者公共使用数据库中所有年龄<18 岁接受 CHD 手术的患者进行了查询。比较了 DS 组和 NS 组之间的院内死亡率、住院时间和费用，根据 CHD 手术进行分层。在 18 年期间，2841 例 DS 患儿接受了 CHD 手术，而 25063 例 NS 患儿接受了 CHD 手术。DS 患儿最常见的干预类型是 AVSD 修复、孤立性室间隔缺损（VSD）修复和法洛四联症（TOF）修复。通过多变量分析，与 NS 患儿相比，DS 患儿在孤立性 AVSD 修复后死亡率较低（RR0.40[IQR0.20-0.79]），在双向 Glenn 吻合术（BDG）（RR5.17[IQR2.10-12.77]）和 TOF/肺动脉瓣闭锁修复（RR9.71[IQR2.16-43.68]）后死亡率较高。使用倾向评分匹配也得到了类似的结果。与 NS 患儿相比，DS 患儿在 AVSD 修复后死亡率较低，但在 BDG 和 TOF/肺动脉瓣闭锁修复后死亡率较高。需要进一步研究以确定 DS 患儿中肺动脉高压的存在是否根据心脏病变改变 DS 与死亡率之间的关联。
