先天性心脏病患儿植入心室辅助装置的结局：对机械循环支持儿科机构间登记系统（Pedimacs）的分析。
Outcomes of children with congenital heart disease implanted with ventricular assist devices: An analysis of the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs).
机构信息
Department of Pediatrics, Division of Pediatric Cardiology, University of Michigan, Ann Arbor, Michigan, USA.
Kirklin Institute for Research in Surgical Outcomes, University of Alabama at Birmingham, Birmingham, Alabama, USA.
出版信息
J Heart Lung Transplant. 2019 Apr;38(4):420-430. doi: 10.1016/j.healun.2018.10.008. Epub 2018 Oct 31.
BACKGROUND
The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children.
METHODS
Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device.
RESULTS
Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 ± 5.7 years vs 9.8 ± 6.5 years; p < 0.0001) and smaller (0.8 ± 0.5 m vs 1.2 ± 0.7 m; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02).
CONCLUSIONS
VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with CHD who received implantable continuous-flow VADs, survival rates were higher and comparable to those of children without CHD. Increased experience correlated with better survival in pediatric VADs.
背景
目前有关儿科先天性心脏病（CHD）患者心室辅助装置（VAD）的报告经验有限。我们旨在描述 VAD 在 CHD 患儿中的应用现状和结局，并与非 CHD 患儿的结局进行比较。
方法
纳入 2012 年 9 月 19 日至 2017 年 6 月 30 日期间登记在儿科机械循环支持机构间注册系统（Pedimacs）中的患者。CHD 分为双心室（第 1、2 或 3 期）和单心室。比较两组患者的结局，采用多变量分析确定与 VAD 相关死亡率的因素。
结果
在纳入的 471 例患者中，108 例（24%）患有 CHD（45 例双心室和 63 例单心室）。与非 CHD 患者相比，CHD 患者更年轻（5.7±5.7 岁 vs. 9.8±6.5 岁；p＜0.0001），体型更小（0.8±0.5 m vs. 1.2±0.7 m；p＜0.0001）。与非 CHD 患者相比，CHD 患者更倾向于接受体外连续流 VAD（36.1% vs. 12.9%；p＜0.0001），而较少接受植入式连续流 VAD（27.8% vs. 55.0%；p＜0.0001）。VAD 治疗 6 个月后，CHD 患者的死亡率（36.4% vs. 12.1%）和移植率（29.1% vs. 59.9%）均高于非 CHD 患者（p＜0.0001）。多变量分析显示，CHD 是与 VAD 死亡率相关的最强因素（风险比 [HR] = 2.9；p＜0.0001），而植入式连续流装置和大容量中心是保护性因素（HR = 0.3，p＜0.0001 和 HR = 0.6，p = 0.02）。
结论
与非 CHD 患儿相比，CHD 患儿使用 VAD 后死亡率增加，移植率降低。对于接受植入式连续流 VAD 的 CHD 患儿亚组，生存率更高，与非 CHD 患儿相当。经验的增加与儿科 VAD 患者的生存率提高相关。
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