小儿致心律失常性右室心肌病诊断及治疗分析  

Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children

作　　者：王树水[1] 张智伟[1] 徐衍梅[1] 蒋秋平[1] 李虹[1] 钱明阳[1] 李渝芬[1] 

机构地区：[1]广东省心血管病研究所广东省人民医院广东省医学科学院心儿科,广东广州510080

出　　处：《中国当代儿科杂志》2010年第3期165-168,共4页Chinese Journal of Contemporary Pediatrics

摘　　要：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。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 obvious impaired ventricular function, additional pharmacological therapy including vasodilators, diuretics, and digitalis were given.Results Ventricular extrasystoles occurred in all 14 cases and ventricular tachycardia in 8 cases. Ten cases showed Epsilon wave on electrocardiography. All 14 cases had enlarged right ventricle and reduced right ventricular ejection fraction. CT or MRI examination showed right ventricular dilatation and a thinned wall of right ventricle in 10 cases. Ventricular extrasystoles or tachycardia disappeared in 7 cases and was reduced in 4 cases after treament. The two children receiving catheter ablation treatment did not present ventricular extrasystoles or tachycardia in a 3-month follow-up. The heart function was improved in the 6 children with obvious impaired ventricular function after pharmacological therapy.Conclusions The clinical manifestations are diverse in children with ARVC. A definite diagnosis of ARVC should be based on a combination of electrocardiography and echocardiography examinations. Pharmacological therapy is effective partially. Catheter ablation treatment appears to be a promising option in patients with drug-refractory ventricular extrasystoles.

关 键 词：致心律失常性右室心肌病 室性心动过速 抗心律失常药物 导管消融 儿童 

分 类 号：R725.4[医药卫生—儿科]