小儿致心律失常性右室心肌病诊断及治疗分析

目的：总结小儿致心律失常性右室心肌病的诊断及治疗体会。方法：14例致心律失常性右室心肌病患儿，男7例，女7例，年龄3~14岁，予心电学、心脏超声、CT、磁共振等检查。分别予胺碘酮+普萘洛尔、索他洛尔治疗，其中2例药物治疗无效者予以导管射频消融治疗。6例心功能减退明显者予以强心、利尿及扩血管治疗。结果：14例均有频发室性早搏，8例见室性心动过速发作。10例检出Epsilon波。14例患儿均有右室扩大及右室射血分数减低。5例行CT检查及5例行磁共振检查患儿均可见到右室扩张、右室室壁变薄。经治疗7例未再出现室性早搏及室性心动过速，4例室性早搏及室性心运过速发作减少。2例予以射频消融治疗的患儿随诊3月未发现室性早搏及室性心动过速发作。心功能不全患儿经药物治疗病情改善。结论：致心律失常性右室心肌病临床表现多样，需综合心电学、心脏超声等多项检查才能临床确诊。药物治疗只对部分病例有效，射频消融治疗有望在室性心律失常治疗中发挥作用。［中国当代儿科杂志，2010，12（3）：165-168］

Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children

Objective To summarize the experience in diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy (ARVC) in children.Methods A total of 14 children (7 females and 7 males) with ARVC were involved. The cases underwent electrocardiography, echocardiography, cardiac CT or MRI examinations. All cases were treated with sotalol or amiodarone in combination with propranolol. In 2 cases with drug-refractory ventricular extrasystoles, catheter ablation treatment was performed. In 6 cases with obvio...