小儿左室心肌致密化不全患者的临床表型和预后分析  

Clinical Features and Outcomes in Childhood Left Ventricular Non-compaction Cardiomyopathy

作　　者：徐立武 陆敏杰[2] 杨凯 吴桂鑫 罗晓亮 XU Liwu;LU Minjie;YANG Kai;WU Guixin;LUO Xiaoliang(Putian No.95 Hospital of China Rongtong Medical Health Group,Putian 351100,Fujian,China;State Key Laboratory of Cardiovascular Disease,Fuwai Hospital,Chinese Academy of Medical Science&Peking Union Medical College Hospital,Beijing 100037,China)

机构地区：[1]中国融通医疗健康集团莆田九十五医院,福建莆田351100 [2]国家心血管病中心北京协和医学院中国医学科学院阜外医院,北京市100037

出　　处：《中国分子心脏病学杂志》2023年第3期5410-5415,共6页Molecular Cardiology of China

基　　金：中央高校基本科研业务费(3332022017)。

摘　　要：目的探讨小儿左室心肌致密化不全(left ventricular non-compaction cardiomyopathy,LVNC)患者的临床表型和预后的影响因素。方法回顾性分析2015年6月至2022年3月在中国医学科学院阜外医院就诊的小儿LVNC患者的临床和随访数据,使用Cox回归分析影响预后因素。结果共纳入69例患者,男54例,女15例,平均年龄(13.7±3.3)岁,平均随访时间(2.1±1.5)年,共13例患者出现心血管病死亡事件。预后生存分析显示心功能Ⅲ-Ⅳ级、肺动脉高压、左房前后径、延迟强化和舒张末期容积指数为小儿LVNC患者心血管病死亡的预测因素。经过多因素Cox校正分析后,舒张末期容积指数为小儿LVNC患者心血管病死亡的独立预测指标(HR=1.028,95%CI 1.004~1.052,P=0.020)。结论舒张末期容积指数是小儿LVNC患者预后的独立预测指标。Objective To investigate the clinical features and outcomes of children with left ventricular non-compaction cardiomyopathy(LVNC).Methods Clinical data and follow-up data of patients with LVNC enrolled in Fuwai hospital were retrospectively analyzed.Risk factors of outcomes were analyzed with Cox regression models.Results A total of 69patients were enrolled with an average age of(13.7±3.3)years at enrollment,including 54 males and 15 females.During(2.1±1.5)years follow-up,13 patients died of cardiovascular events.The Cox univariate analysis showed that cardiac function in class III/IV,pulmonary hypertension,left atrial diameter,late gadolinium enhancement,end-diastolic volume index(EDVi)were predictive factors of cardiovascular death in children with LVNC.Cox multivariate analysis showed that EDVi was independent predictors of cardiovascular death in children with LVNC(HR=1.028,95%CI:1.004-1.052,P=0.020).Conclusions EDVi is an independent predictor of the prognosis of children with LVNC.

关 键 词：小儿 左室心肌致密化不全 临床表型 预后 

分 类 号：R725.4[医药卫生—儿科]