[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 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 treatment. 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.
Wang
Shu-Shui
SS
Department of Pediatric Cardiology, Guangdon Academy of Medical Sciences, Guangdong General Hospital, Guangdong Provincial Cardiovascular Institute, Guangzhou, China.
Zhang
Zhi-Wei
ZW
Xu
Yan-Mei
YM
Jiang
Qiu-Ping
QP
Li
Hong
H
Qian
Ming-Yang
MY
Li
Yu-Fen
YF
D000293:Adolescent; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed