目的　探讨心肌致密化不全（NVM）合并心室电风暴（ES）的诊断和治疗。方法　回顾分析1例NVM合并ES患 儿的临床资料，并复习相关文献。结果　患儿，女， 8岁，近14个月内反复晕厥。彩色多普勒超声心动图示左室心肌致密化 不全，左室收缩功能减退。因恶性室性心律失常反复发作呈ES表现，予以电复律/电除颤，临时心脏起搏以维持血流动力学 稳定，后期植入心脏复律除颤器（ICD）。 术后随访一年内未有阿斯发作，但心脏扩大及心功能未见明显改善，ICD植入术后 14个月发生猝死。结论　NVM为临床少见的心肌病，持续室性心动过速，反复发作，部分呈ES表现，易发生心脏猝死。

Objective　To explore the diagnosis and treatment of noncompaction of ventricular myocardium (NVM) combined with ventricular electrical storm (ES). Methods　The clinical data of a girl with NVM combined with ES for recurrent syncope was retrospectively analyzed, and the relevant literature were reviewed. Results　The female child aged 8 years old was admitted for recurrent syncope within 14 months. Color doppler ultrasonography showed NVM with decreased left ventricle contractile function. Malignant ventricular arrhythmia repeatedly occurred in the form of ES, which was treated with electrocardioversion/ electrodefibrillation, temporary cardiac pacing to maintain hemodynamic stability, and later implantable cardioverter defibrillator (ICD) was implanted. During the one-year follow-up after operation, there was no attack of Adams-Stokes observed, but no significant improvement in cardiac enlargement and cardiac function was reported. Sudden death occurred 14 months after ICD implantation. Conclusion　NVM is a relatively rare type of cardiomyopathy. Ventricular arrhythmia is one of the main clinical manifestations of NVM. Continuous ventricular tachycardia often occurs repeatedly with ES manifestations. Sudden cardiac death may occur.