儿童心肌致密化不全5例临床分析

Clinical features of noncompaction of ventricular myocardium in children

目的分析心肌致密化不全（NVM）患儿的临床特点。方法回顾性分析5例心肌致密化不全患儿的症状、超声心动图、胸部X线、心电图以及治疗和随访资料。结果 5例患儿中,男3例、女2例,年龄3月-12岁;4例以支气管肺炎,心力衰竭入院,5例患儿均有胸部X线心影增大;心脏二维超声示心肌致密化不全累及心尖部2例,累及左心室室壁和室间隔3例;1例伴有先天性部分心内膜垫缺损。5例患儿均无血栓栓塞表现。5例患儿随访3-36月,存活3例,死于顽固性心衰1例,失访1例。结论儿童NVM的主要临床表现为心力衰竭,超声心动图仍是最主要的诊断方法;对于顽固的肺炎心衰心力衰竭应该考虑到NVM的可能。

Objective To assess the clinical features of noncompaction of ventricular myocardium（NVM） in children.Methods The clinical data from five children with NVM were analyzed,including clinical manifestations,ultrasonic cardiogram（UCG）,electrocardiogram,chest X-ray,the process of diagnosis and treatment,and follow up.Results In five patients（3 boys and 2 girls） aged 3 months to 12 years,four of them were hospitalized because of pneumonia with heart failure.None of the five patients had thromboembolism.Heart enlargement was observed in all five patients on chest X-ray.By 2D ultrasonography,2 patients had cardiac apex involvement,and 3 patients showed involvement of left ventricular wall and interventricular septum.In one patient,congenital partial defect of the endocardial cushion was observed.All patients were followed up for 3 to 36 months.One patient died of heart failure,3 patients survived,and one patient was lost to follow-up.Conclusions The common clinical manifestation of NVM is the heart failure.UCG is preferred for NVM diagnosis.In children with refractory pneumonia and heart failure,NVM should be suspected.