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
Department of Pediatric Cardiology, Guangdong Academy of Medical Sciences, Guangdong General Hospital, Guangdong Provincial Cardiovascular Institute, Guangzhou 510080, China. Email:drzhangzhiwei@sina.com
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］
WANG Shu-Shui,ZHANG Zhi-Wei,XU Yan-Mei et al. Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children[J]. 中国当代儿科杂志, 2010, 12(3): 165-168.
WANG Shu-Shui,ZHANG Zhi-Wei,XU Yan-Mei et al. Diagnosis and treatment of arrhythmogenic right ventricular cardiomyopathy in children[J]. CJCP, 2010, 12(3): 165-168.
［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.
［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.
