儿童心肌致密化不全合并先天性心脏病临床分析

Clinical analysis of children with noncompaction of ventricular myocardium combined with congenital heart disease

目的分析并总结心肌致密化不全(NVM)合并先天性心脏病患儿的临床资料,探讨针对该病的最佳治疗方式。方法 2007年9月至2017年6月在该院共搜集48例NVM合并先天性心脏病患儿,并按治疗方式分为介入组(9例)、外科组(11例)和药物组(28例)。分析3组临床表现、孕期情况、既往病史、家族史、辅助检查、治疗方案及预后,比较3组治疗临床疗效。结果 48例患儿中43例肺炎,38例心功能不全,16例心律失常,均无血栓形成,合并的心脏疾病例数从高到低依次为房间隔缺损、肺动脉高压、三尖瓣病变、二尖瓣病变、动脉导管未闭、室间隔缺损。介入组随访6例患儿无NVM,2例好转;外科组随访患儿3例死亡,1例放弃治疗,存活患儿有5例无NVM转;药物组随访患儿中1例死亡,2例放弃治疗,存活患儿中有2例无NVM,9例NVM无明显好转。介入组、外科组患儿手术治疗先天性心脏病后NVM好转率明显高于药物组,差异均有统计学意义(P<0.05)。结论同药物组患儿比较,介入组和外科组患儿治疗先天性心脏病后NVM明显好转。

Objective To analyze and summarize the clinical data of children with myocardial densification （NVM） combined with congenital heart disease, explore the best treatment for the disease. Methods 48 children with NVM combined with congenital heart disease were collected admitted to the hospital fi＇om September 2007 to June 2017. According the treatment plan,the 48 cases divided into 3 groups：interventional gToup（9 cases）, surgical gToup（llcases） and drugs gToup（28 cases）. The clinical manifestations, pregnancy status, previous medical history, family history, auxiliary examination, treatment plan and prognosis of the three groups were analyzed, and the clinical efficacy of the three groups was compared. Results In the 48 cases children, 43 cases had pneumonia, 38 cases had heart failure, 16 cases had arrhythmias, no one had thrombosis.According to the quantity of cases, the combined heart disease were ASD, puhnonary hypertension, the disease of tricuspid valve, the disease of mitral valve, PDA, VSD.In the interventional group, 6 cases had no NVM, 2 case got better.In the surgical group, 3 cases were dead, 1 case was given up, and in the live children, 5 cases had no NVM.In the drugs group, 1 case was dead, 2 cases were given up, and in the live children , 2 cases had no NVM, 9 cases had no significant changes. The improvement rate of NVM after treat- ment for congenital heart disease in the interventional group and the surgical group were significantly higher than that in the drug group, the differences were statistically significant （P〈0.05）. Conclusion Comparing with drugs group, the NVM of the cases of interventional group and surgical group improve obviously after treatment for congenital heart disease.