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

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Chinese Journal of Contemporary Pediatrics ›› 2010, Vol. 12 ›› Issue (3) : 165-168.

CLINICAL RESEARCH

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

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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］

Key words

Arrhythmogenic right ventricular cardiomyopathy / Ventricular tachycardia / Anti-arrhythmia agent / Catheter ablation / Child

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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

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