小儿心肌病和先天性心脏病中的心室辅助装置：德国先天性心脏缺陷国家登记处的分析。

Ventricular assist devices in paediatric cardiomyopathy and congenital heart disease: An analysis of the German National Register for Congenital Heart Defects.

机构信息

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Germany; Department of Paediatric Cardiology, University Hospital Muenster, Germany; Competence Network for Congenital Heart Defects Berlin, Germany.

Department of Paediatric Cardiology, University Hospital Muenster, Germany.

出版信息

Int J Cardiol. 2021 Nov 15;343:37-44. doi: 10.1016/j.ijcard.2021.08.047. Epub 2021 Sep 3.

DOI:10.1016/j.ijcard.2021.08.047

PMID:34487787

Abstract

BACKGROUND

Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children.

METHODS

All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected.

RESULTS

Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000-2004 to 27 implantations in the time period 2010-2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p < 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality.

CONCLUSIONS

The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation.

摘要

背景

由于获得性心脏病，心室辅助装置（VAD）越来越多地用于终末期心力衰竭患者。关于该技术在儿童中的使用和结果的数据有限。

方法

在德国先天性心脏病国家登记处中确定了所有接受 VAD 支持的儿童（<18 岁），并收集了人口统计学，基础心脏缺陷，先前的手术，相关条件，手术类型，并发症和结果的数据。

结果

在 8 家德国中心，总共纳入了 64 名（中位年龄 2.1 岁；女性占 45.3%）在 1999 年至 2015 年间接受 VAD 的患者。基础诊断为先天性心脏病（CHD）的有 25 例，心肌病的有 39 例。报告的 VAD 植入数量从 2000-2004 年期间的 13 例增加到 2010-2014 年期间的 27 例。在 VAD 支持的中位数为 54 天期间，28.1%的患者出现出血并发症（6.3%为颅内出血），14.1%出现血栓形成（10.9%为 VAD 血栓形成）和 23.4%的血栓栓塞并发症（包括 18.8%的患者发生脑梗死）。与 CHD 患者相比，心肌病患者更有可能接受心脏移植（79.5%比 28.0%）。与 CHD 队列相比，心肌病患者的生存率明显更好（p<0.0001）。多变量 Cox 比例风险分析显示，CHD 诊断（风险比[HR]4.04，p=0.001），VAD 植入时的年龄（HR 1.09/年，p=0.04）和 VAD 植入前需要体外膜氧合（ECMO）支持（HR 3.23，p=0.03）是死亡率的独立预测因素。