【摘要】 目的探讨小儿孤立性心肌致密化不全(NCCM)的临床特点。方法回顾性分析13例NCCM病儿的临床资料。结果 13例病儿均未出现栓塞表现,但有明显心功能不全者8例,心律失常者12例,其中室性期前收缩4例。13例病儿均可见典型的NCCM超声心动图改变,其中左心室单独受累12例,双心室受累1例;左心室增大9例,左心室排血分数降低8例;肺动脉收缩压增高9例。随访0.5～4.4年,1例猝死,2例死于顽固性心力衰竭,3例因心力衰竭多次住院。结论小儿NCCM以心功能不全及心律失常多见,栓塞事件罕见,超声心动图对NCCM的诊断具有重要价值,NCCM预后差。更多还原

【Abstract】 Objective To investigate the clinical characteristics of isolated non-compaction cardiomyopathy(NCCM) in children.Methods Clinical data of 13 NCCM children were analyzed retrospectively.Results There was no manifestations of embolism presented in all the 13 NCCM children,but eight of them had obvious cardiac dysfunction,12 had cardiac arrhythmia,of whom,four had premature ventricular beats.Typical echocardiographic changes were observed in all the 13 children,of whom,12 were involved in the left ventricle alone,both ventricles involved in one;left ventricular enlargement was seen in nine,eight with decreased left ventricular ejection fraction,and nine had an increased pulmonary artery systolic pressure.During 0.5-4.4-year follow-up,sudden death was noted in one case,two died of intractable heart failure;and three repeated hospitalization due to heart failure.Conclusion Cardiac dysfunction and arrhythmia are frequently observed in children with NCCM,and embolism is rare.Ultrasound cardiography is of great value in the diagnosis of NCCM——a disease of poor prognosis.更多还原