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左心室心肌致密化不全——儿科人群危险因素的系统评价

Left Ventricular Noncompaction-A Systematic Review of Risk Factors in the Pediatric Population.

作者信息

Łuczak-Woźniak Katarzyna, Werner Bożena

机构信息

Department of Pediatric Cardiology and General Pediatrics, Doctoral School, Medical University of Warsaw, 02-091 Warsaw, Poland.

Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw, 02-091 Warsaw, Poland.

出版信息

J Clin Med. 2021 Mar 16;10(6):1232. doi: 10.3390/jcm10061232.

Abstract

Left ventricular noncompaction (LVNC) is a heterogeneous, often hereditary group of diseases, which may have diverse clinical manifestations. This article reviews the risk factors for unfavorable outcomes of LVNC in children, as well as discuss the diagnostic methods and the differences between pediatric and adult LVNC. Through a systematic review of the literature, a total of 1983 articles were outlined; 23 of them met the inclusion criteria. In echocardiography the following have been associated with adverse outcomes in children: Left ventricular ejection fraction, end-diastolic dimension, left ventricular posterior wall compaction, and decreased strains. T-wave abnormalities and increased spatial peak QRS-T angle in ECG, as well as arrhythmia, were observed in children at greater risk. Cardiac magnetic resonance is a valuable tool to identify those with systolic dysfunction and late gadolinium enhancement. Genetic testing appears to help identify children at risk, because mutations in particular genes have been associated with worse outcomes. ECG and imaging tests, such as echocardiography and magnetic resonance, help outline risk factors for unfavorable outcomes of LVNC in children and in identifying outpatients who require more attention. Refining the current diagnostic criteria is crucial to avoid inadequate restrain from physical activity.

摘要

左心室心肌致密化不全(LVNC)是一组异质性疾病,通常具有遗传性,临床表现多样。本文综述了儿童LVNC不良预后的危险因素,并讨论了诊断方法以及儿童与成人LVNC的差异。通过系统的文献回顾,共筛选出1983篇文章;其中23篇符合纳入标准。在超声心动图检查中,以下因素与儿童不良预后相关:左心室射血分数、舒张末期内径、左心室后壁致密化以及应变降低。心电图显示T波异常、空间QRS-T角增大以及心律失常在高危儿童中更为常见。心脏磁共振成像对于识别收缩功能障碍和晚期钆增强患者是一种有价值的工具。基因检测似乎有助于识别高危儿童,因为特定基因的突变与更差的预后相关。心电图以及超声心动图和磁共振成像等影像学检查有助于明确儿童LVNC不良预后的危险因素,并识别需要更多关注的门诊患者。完善当前的诊断标准对于避免不适当限制体育活动至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2653/8001197/1d274389868d/jcm-10-01232-g001.jpg

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