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重症心力衰竭以及患有心肌炎的儿科患者对机械循环支持和心脏移植的需求:来自前瞻性多中心注册研究“MYKKE”的结果

Severe heart failure and the need for mechanical circulatory support and heart transplantation in pediatric patients with myocarditis: Results from the prospective multicenter registry "MYKKE".

作者信息

Schubert Stephan, Opgen-Rhein Bernd, Boehne Martin, Weigelt Annika, Wagner Robert, Müller Götz, Rentzsch Axel, Zu Knyphausen Edzard, Fischer Marcus, Papakostas Konstantin, Wiegand Gesa, Ruf Bettina, Hannes Tobias, Reineker Katja, Kiski Daniela, Khalil Markus, Steinmetz Michael, Fischer Gunther, Pickardt Thomas, Klingel Karin, Messroghli Daniel R, Degener Franziska

机构信息

Department of Congenital Heart Disease - Pediatric Cardiology, German Heart Center Berlin, Berlin, Germany.

DZHK (German Centre for Cardiovascular Research), Berlin, Germany.

出版信息

Pediatr Transplant. 2019 Nov;23(7):e13548. doi: 10.1111/petr.13548. Epub 2019 Jul 11.

Abstract

Myocarditis represents an important cause for acute heart failure. MYKKE, a prospective multicenter registry of pediatric patients with myocarditis, aims to gain knowledge on courses, diagnostics, and therapy of pediatric myocarditis. The role of mechanical circulatory support (MCS) in children with severe heart failure and myocarditis is unclear. The aim of this study was to determine characteristics and outcome of patients with severe heart failure requiring MCS and/or heart transplantation. The MYKKE cohort between September 2013 and 2016 was analyzed. A total of 195 patients were prospectively enrolled by 17 German hospitals. Twenty-eight patients (14%) received MCS (median 1.5 years), more frequently in the youngest age group (0-2 years) than in the older groups (P < 0.001; 2-12 and 13-18 years). In the MCS group, 50% received a VAD, 36% ECMO, and 14% both, with a survival rate of 79%. The weaning rate was 43% (12/28). Nine (32%) patients were transplanted, one had ongoing support, and six (21%) died. Histology was positive for myocarditis in 63% of the MCS group. Patients within the whole cohort with age <2 years and/or ejection fraction <30% had a significantly worse survival with high risk for MCS, transplantation, and death (P < 0.001). Myocarditis represents a life-threatening disease with an overall mortality of 4.6% in this cohort. The fulminant form more often affected the youngest, leading to significantly higher rate of MCS, transplantation, and mortality. MCS represents an important and life-saving therapeutic option in children with myocarditis with a weaning rate of 43%.

摘要

心肌炎是急性心力衰竭的一个重要病因。MYKKE是一项针对小儿心肌炎患者的前瞻性多中心注册研究,旨在了解小儿心肌炎的病程、诊断和治疗。机械循环支持(MCS)在重症心力衰竭和心肌炎患儿中的作用尚不清楚。本研究的目的是确定需要MCS和/或心脏移植的重症心力衰竭患者的特征和预后。对2013年9月至2016年期间的MYKKE队列进行了分析。17家德国医院前瞻性纳入了195例患者。28例患者(14%)接受了MCS(中位时间1.5年),最年轻年龄组(0 - 2岁)比年长组更频繁接受MCS(P < 0.001;2 - 12岁和13 - 18岁)。在MCS组中,50%接受了心室辅助装置(VAD),36%接受了体外膜肺氧合(ECMO),14%两者都接受,生存率为79%。撤机率为43%(12/28)。9例(32%)患者接受了心脏移植,1例仍在接受支持治疗,6例(21%)死亡。MCS组中63%的患者组织学检查显示心肌炎阳性。整个队列中年龄<2岁和/或射血分数<30%的患者生存率显著更差,接受MCS、心脏移植和死亡的风险更高(P < 0.001)。心肌炎是一种危及生命的疾病,本队列中的总体死亡率为4.6%。暴发性心肌炎更常影响最年幼的患儿,导致MCS、心脏移植和死亡率显著更高。MCS是心肌炎患儿重要的救命治疗选择,撤机率为43%。

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