Divisions of Pediatric Cardiology, University of Arkansas Medical Center, Little Rock, Arkansas, USA.
ASAIO J. 2013 Jan-Feb;59(1):52-6. doi: 10.1097/MAT.0b013e31827b5a45.
Our objective was to evaluate morbidity and mortality associated with extracorporeal membrane oxygenation (ECMO) in children with genetic syndromes and heart disease. We conducted a retrospective review of all children with heart disease and genetic syndromes receiving ECMO during the period January 2000 and March 2012 at Arkansas Children's Hospital, Little Rock. The medical charts were reviewed to obtain the following variables: demographic information, medical and surgical history, laboratory and microbiological, information on organ dysfunction, and outcome characteristics. The outcome variables evaluated in this report included: hospital length of stay (LOS), survival to hospital discharge, and current survival. Outcome data were compared among critically ill children with and without syndromes. During the study period, there were 377 ECMO runs in 336 children with heart disease. Of these, 43 ECMO runs occurred in children with genetic syndromes whereas 334 ECMO runs occurred in children with no genetic abnormality. Children in the group with underlying genetic syndrome were older at the time of ECMO cannulation than the group with no syndrome. During the ECMO run, hospital LOS and mortality were similar in children with and without underlying genetic abnormality. Among genetically abnormal patients, renal insufficiency and need for dialysis were associated with mortality. In this group, 24 patients (56%) were discharged alive. However, only 10 patients are living to date in this cohort. ECMO can be used in children with heart disease and genetic syndromes with good results. The survival rate is high and the complication rate is low.
我们的目的是评估患有遗传性综合征和心脏病的儿童使用体外膜肺氧合（ECMO）的发病率和死亡率。我们对 2000 年 1 月至 2012 年 3 月期间在阿肯色州儿童医院接受 ECMO 治疗的所有患有心脏病和遗传性综合征的儿童进行了回顾性研究。回顾了病历以获取以下变量：人口统计学信息、医疗和手术史、实验室和微生物学信息、器官功能障碍信息以及预后特征。本报告评估的预后变量包括：住院时间（LOS）、存活至出院和当前存活。比较了危重症患儿有无综合征的预后。在研究期间，有 336 例心脏病患儿进行了 377 次 ECMO 运行。其中，43 次 ECMO 运行发生在患有遗传性综合征的儿童中，334 次 ECMO 运行发生在没有遗传异常的儿童中。在 ECMO 插管时，患有潜在遗传综合征的患儿比无综合征的患儿年龄更大。在 ECMO 运行期间，有潜在遗传异常的患儿和无遗传异常的患儿的住院 LOS 和死亡率相似。在遗传异常的患者中，肾功能不全和需要透析与死亡率相关。在这组患者中，24 名患者（56%）存活出院。然而，在这组患者中，目前仅有 10 名患者存活。ECMO 可用于患有心脏病和遗传性综合征的儿童，效果良好。存活率高，并发症发生率低。
