小儿致心律失常性右室心肌病诊断及治疗分析

王树水; 张智伟; 徐衍梅; 蒋秋平; 李虹; 钱明阳; 李渝芬

PDF(1232 KB)

中国当代儿科杂志 ›› 2010, Vol. 12 ›› Issue (3) : 165-168.

论著·临床研究

小儿致心律失常性右室心肌病诊断及治疗分析

王树水，张智伟，徐衍梅，蒋秋平，李虹，钱明阳，李渝芬

作者信息 +

Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children

WANG Shu-Shui, ZHANG Zhi-Wei, XU Yan-Mei, JIANG Qiu-Ping, LI Hong, QIAN Ming-Yang, LI Yu-Fen

Author information +

文章历史 +

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。［中国当代儿科杂志，2010，12（3）：165-168］

Abstract

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.［Chin J Contemp Pediatr, 2010, 12 (3):165-168］

导出引用

王树水, 张智伟, 徐衍梅, 蒋秋平, 李虹, 钱明阳, 李渝芬. 小儿致心律失常性右室心肌病诊断及治疗分析[J]. 中国当代儿科杂志. 2010, 12(3): 165-168

WANG Shu-Shui, ZHANG Zhi-Wei, XU Yan-Mei, JIANG Qiu-Ping, LI Hong, QIAN Ming-Yang, LI Yu-Fen. Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children[J]. Chinese Journal of Contemporary Pediatrics. 2010, 12(3): 165-168

中图分类号： R725.4

参考文献

［1］Peters S. Advances in the diagnostic management of arrhythmogenic right ventricular dysplasia/cardiomyopathy［J］. Int J Cardiol, 2006, 113(1):4-11.
［2］Wong AR, AbdurRazak N, Al-Hadlaq SM, Al-Jarallah AS. Arrhythmogenic right ventricular cardiomyopathy in an 11-year-old girl and typical echocardiographic features［J］. Pediatr Cardiol, 2008, 299(2):427-430.
［3］Nucifora G, Benettoni A, Allocca G, Bussani R, Silvestri F. Arrhythmogenic right ventricular dysplasia/cardiomyopathy as a cause of sudden infant death［J］. J Cardiovasc Med, 2008, 9(4):430-431.
［4］McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, et al. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology［J］. Br Heart J, 1994, 71(3):215-218.
［5］Peters S, Trummel M, Koehler B, Westermann KU. The value of different electrocardiographic depolarization criteria in the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy［J］. J Electrocardiol, 2007, 40(1):34-37.
［6］吴书林. 致心律失常性右室心肌病心电图诊断进展［J］. 临床心电学杂志, 2007, 16(5):377-380.
［7］Francés RJ. Arrhythmogenic right ventricular dysplasia/cardiomyopathy. A review and update［J］. Int J Cardiol, 2006, 110(3):279-287.
［8］Kimura F, Sakai F, Sakomura Y, Fujimura M, Ueno E, Matsuda N, et al. Helical CT features of arrhythmogenic right ventricular cardiomyopathy［J］. Radiographics, 2002, 22(5):1111-1124.
［9］Yoerger DM, Marcus F, Sherrill D, Calkins H, Towbin JA, Zareba W, et al. Echocardiographic findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasia: new insights from the multidisciplinary study of right ventricular dysplasia［J］. J Am Coll Cardiol, 2005, 45(6):860-865.
［10］van der Wall EE, Kayser HW, Bootsma MM, de Roos A, Schalij MJ. Arrhythmogenic right ventricular dysplasia: MRI findings ［J］. Herz, 2000, 25(4):356-364.
［11］Maintz D, Juergens KU, Grude M, Ozgun M, Fischbach R, Wichter T. Images in cardiovascular medicine. Magnetic resonance imaging and computed tomography findings in arrhythmogenic right ventricular cardiomyopathy［J］. Circulation, 2006, 113(13):e673-675.
［12］Wichter T, Paul TM, Eckardt L, Gerdes P, Kirchhof P, B-cker D, et al. Arrhythmogenic right ventricular cardiomyopathy antiarrhythmic drugs, catheter ablation, or ICD?［J］ .Herz, 2005, 30(2):91-101.
［13］Cox MG, Nelen MR, Wilde AA, Wiesfeld AC, van der Smagt JJ, Loh P, et al. Activation delay and VT parameters in arrhythmogenic right ventricular dysplasia/cardiomyopathy: toward improvement of diagnostic ECG criteria［J］. J Cardiovasc Electrophysiol, 2008, 19(8):775-813.