儿童心肌致密化不全5例临床分析

摘要

ObjectiveTo assess the clinical features of noncompaction of ventricular myocardium (NVM) in children. Methods The clinical data from ifve children with NVM were analyzed, including clinical manifestations, ultrasonic cardio-gram(UCG), electrocardiogram, chest X-ray, the process of diagnosis and treatment, and follow up.Results In ifve 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 ifve patients had thromboembolism. Heart enlargement was observed in all ifve 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.%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。