目的 儿童左室致密化不全（LVNC）临床表现缺乏特异性，目前关于LVNC预后危险因素的系统性研究相对较少，临床尚缺乏明确的风险分层依据。本研究旨在分析儿童LVNC的临床特征、基因变异及预后影响因素。方法 回顾性分析2018年6月至2024年6月确诊的42例LVNC患儿的临床资料。依据随访期间是否发生心血管不良事件分为不良事件组（15例）与无不良事件组（27例），比较两组间临床和实验室指标，并采用多因素logistic回归分析预后危险因素。结果 42例患儿中男性24例（57.1%），确诊中位年龄92.5月龄。常见首诊症状为纳差伴气促（19.0%）、胸闷胸痛（19.0%）及心悸（16.7%）。首诊时心功能Ⅲ/Ⅳ级患儿35.7%（15/42），心电图异常92.9%（39/42），以快速型心律失常最常见（14/42）。超声心动图示左房、左室增大分别占71.4%（30/42）和80.9%（34/42），左室射血分数（LVEF）中位数为52.0%（41.8%~62.0%），疏松层/致密层（NC/C）比值中位数2.5（2.1~3.0）。4 例患儿有一级亲属猝死或心肌病家族史；16例患儿接受基因检测，其中10例检出致病性基因变异。中位随访30个月，4例患儿死亡，病死率9.5%；45.2%（19/42）的患儿症状改善。不良事件组快速型心律失常发生率、心功能Ⅲ/Ⅳ级比例及NC/C比值均显著高于无不良事件组（P<0.05）。多因素logistic回归分析显示，心功能Ⅲ/Ⅳ级（OR=13.55，95% CI：1.87~98.39，P=0.01）、快速型心律失常（OR=19.73，95%CI：2.28~170.47，P=0.007）、NC/C比值升高（OR=5.19，95% CI：1.20~22.40，P=0.027）是预后不良的独立危险因素。结论 儿童LVNC临床表现异质性强，常伴遗传背景；心功能差、快速型心律失常及高NC/C比值为预后不良的独立危险因素，临床需加强对高危患儿的监测与干预。

Objective Clinical manifestations of left ventricular noncompaction (LVNC) in children lack specificity. At present, there are relatively few systematic studies on the prognostic risk factors of LVNC, and clear evidence for risk stratification is still lacking in clinical practice. This study aimed to analyze the clinical characteristics, genetic variations, and prognostic factors of LVNC in children. Methods Clinical data of 42 children diagnosed with LVNC from June 2018 to June 2024 were retrospectively analyzed. According to the occurrence of adverse cardiovascular events during follow-up, they were divided into an adverse event group (15 cases) and a non-adverse event group (27 cases). Clinical and laboratory indicators were compared between the two groups, and multivariate Logistic regression was used to analyze prognostic risk factors. Results Among the 42 children, 24 were males (57.1%), with a median age of 92.5 months at diagnosis. The common initial symptoms were poor appetite accompanied by shortness of breath (19.0%), chest tightness or chest pain (19.0%), and palpitations (16.7%). At the initial diagnosis,children with cardiac function class III/IV accounted for 35.7% (15/42).92.9% (39/42) of the children had abnormal electrocardiograms (ECG), with tachyarrhythmia being the most common (14/42). Echocardiography revealed left atrial and left ventricular enlargement in 71.4% (30/42) and 80.9% (34/42) of patients, respectively. The median left ventricular ejection fraction (LVEF) was 52.0% (41.8-62.0), and the median non-compacted to compacted (NC/C) ratio was 2.5 (2.1-3.0). Four children had a family history of sudden death or cardiomyopathy in first-degree relatives. 16 children underwent genetic testing, and pathogenic gene mutations were detected in 10 cases. The median follow-up duration was 30 months, the fatality rate was 9.5%, and symptoms improved in 45.2% (19/42) of children. The group with adverse events exhibited a significantly higher incidence of tachyarrhythmia, a greater proportion of patients with cardiac function class III/IV, and higher NC/C ratios compared to the group without adverse events (P<0.05). Multivariate logistic regression analysis demonstrated that cardiac function class Ⅲ/IV (OR=13.55, 95% CI: 1.87~98.39, P= 0.01), tachyarrhythmia (OR=19.73, 95% CI: 2.28~170.47, P=0.007), and an elevated NC/C ratio (OR=5.19, 95% CI: 1.20~22.40, P=0.027) as independent risk factors for an unfavorable prognosis. Conclusions Children with LVNC exhibit strong heterogeneity in clinical manifestations and are often associated with a genetic background. Poor cardiac function, tachyarrhythmia, and a high NC/C ratio are independent risk factors for poor prognosis, so intensive monitoring and intervention for highrisk children are required in clinical practice.