探讨超声心动图对儿童心内膜弹力纤维增生症合并心肌致密化不全的诊断价值

摘要

目的 探讨超声心动图对于儿童心内膜弹力纤维增生症(EFE)合并心肌致密化不全(LVNC)的临床诊断价值.方法 回顾性分析我院收治EFE合并LVNC患儿(A组)的临床表现及辅助检查,并选取此期间特发性EFE患儿(B组)作对比.两组患儿均按EFE的治疗方案进行治疗,并对其进行随访.对两组患儿进行比较,并做出归纳和总结.结果 两组患儿在发病时心内膜厚度和左室射血分数(LVEF)不具有统计学差异(P＞0.05).经过随访,两组患儿心内膜厚度恢复情况不具有统计学差异(P＞0.05),但A组患儿LVEF中(42.50±8.73 vs 50.50±6.61,P＜0.05)、远(46.17±5.12 vs 55.40士9.12,P＜0.05)期恢复情况低于B组.结论 EFE合并LVNC患儿与特发性EFE患儿相比,中远期预后较差.对于EFE、LVNC的诊断和观察,超声心动图为首选检查手段.%Objective To investigate the clinical diagnostic value of echocardiography in pediatric patients of EFE combined with LVNC.Methods A retrospective analysis of the clinical presentation and supplementary examination in our hospital was conducted about children of EFE combined with LVNC(Group A),and made contrast with the children of idiopathic EFE(Group B).Two groups of children accepted the same treatment according to EFE,and made follow-up with them.Compared the indicators between two groups,and then made generalization and summary of these results.Results Echocardiography showed no significant difference in endocardial thickness and LVEF between two groups at initial(P＞0.05).After follow-up,the recovery of the endocardial thickness had no difference(P＞0.05).But the recovery of LVEF in Group A was worse(P＜0.05) than in Group B at medium(42.50±8.73 vs 50.50± 6.61,P＜0.05) and long-term(46.17±5.12 vs 55.40±9.12,P＜0.05) follow-up.Conclusions The medium and long-term prognosis in pediatric patients of EFE combined with LVNC was worse than those of idiopathic EFE.Echocardiograpy was an initial method and had special advantages in diagonsis and observation of EFE and LVNC.