目的　探讨不同程度心肌致密化不全患儿的临床特征及预后，探讨非致密化心肌层与致密心肌层厚度之比 （N/C）的临床指导意义。方法　回顾分析心肌致密化不全患儿的临床资料，比较N/C>2.0和N/C 1.4~2.0两组患儿的情况。 结果　两组患儿各26例，年龄、性别、低出生体质量和营养不良比例，心脏病变及合并症的差异均无统计学意义（P>0.05）。 出院时N/C>2.0组病情好转8例，无明显好转15例，死亡3例；N/C 1.4~2.0组病情好转18例，无明显好转8例，无死亡病 例。两组治疗有效率差异有统计学意义（χ2=7.69， P=0.006）。 随访出院后8年内N/C>2.0组病死率高于N/C 1.4~2.0组， 差异有统计学意义（χ2=6.993， P=0.008）。 结论　心肌致密化不全患儿的N/C值越大，治疗有效率越低，预后越差。

　Objectives　To explore the clinical characteristics and prognosis of different degrees of noncompaction of ventricular myocardium (NVM) in children and to investigate the clinical significance of the ratio of the thickness of the noncompact myocardium to the dense myocardium (N/C). Methods　The clinical data of the children with NVM were analyzed retrospectively. The differences between children with N/C > 2.0 and children with N/C 1.4~2.0 were compared. Results　There were 26 children in each of the two groups. There was no significant difference in age, sex, the ratios of low birth weight and malnutrition, heart disease, and complication (P all > 0.05). When discharged, 8 cases were improved, 15 cases has no obvious improvement, and 3 cases died in N/C>2.0 group; while 18 cases were improved, 8 cases has no obvious improvement, and none died in N/C 1.4~2.0 group, and the effective rate of treatment between two groups was statistically difference (χ2=7.69， P=0.006). In 8 years of follow-up, the mortality rate in N/C > 2.0 group was higher than that in N/C 1.4~2.0 group, and the difference was statistically different (χ2=6.993, P=0.008). Conclusions　The treatment efficiency was lower and the prognosis was worse when the N/C value was higher in children with NVM.