摘要： 心肌致密化不全又称海绵状心肌病，大部分患儿以左心室心肌致密化不全 （LVNC） 为主。LVNC是一种与基因及遗传有关的心肌发育异常性疾病，突出的肌小梁和深陷的隐窝为其主要病理改变。随着临床医学的发展，LVNC的诊断率逐渐增加，由于该病预后较差，在临床上也逐渐受到关注。日前，临床以超声心动图影像学表现作为诊断的主要手段，具体诊断还应结合患儿的临床特征。LVNC的治疗以对症治疗为主，主要目标为改善心肌质量，提高患儿生活质量。本文针对LVNC的临床特征、诊断方法、目前可行的治疗手段及不同患者预后情况进行综述，旨在对LVNC的诊治及预后提供参考。

儿童左心室心肌致密化不全诊治的研究进展

李栋, 王策

中国医科大学附属盛京医院小儿心脏科, 沈阳 110004

收稿日期:2022-09-08出版日期:2023-05-30发布日期:2023-05-26
通讯作者:王策E-mail:crease.sy@163.com
作者简介:李栋 (1999-),男,硕士研究生.
基金资助:国家自然科学基金(82001598)；辽宁省自然科学基金(2020-BS-090)


关键词: 心肌病, 左心室心肌致密化不全, 儿童
Abstract: Non-compaction cardiomyopathy,also known as cavernous cardiomyopathy,is dominated by left ventricular non-compaction (LVNC) in most patients. LVNC is an inherited genetic disease causing myocardial abnormality. The main pathological features of non-compaction cardiomyopathy are a large number of prominent trabeculae and deep crypts in the ventricular wall. With the development of clinical medicine,the diagnosis rate of LVNC in children is gradually increasing and,because of its poor prognosis,the disease has gained increasing attention in clinics. At present,the clinical diagnosis is mainly based on echocardiographic imaging findings but the specific diagnosis should also be based on the patient's clinical characteristics. The treatment of LVNC,mainly symptomatic treatment,should be carried out according to the clinical manifestations of individual patients. The main goal is to improve myocardial quality and improve patients' quality of life. This paper reviewed recent research on the clinical features,diagnostic methods,feasible treatment methods and prognosis of different patients with non-compaction of the myocardium,especially in children. This review aims to provide ideas for clinical diagnosis,treatment and prognosis prediction of LVNC in children.
Key words: cardiomyopathy, left ventricular non-compaction, child
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