Arrhythmogenic right ventricular dysplasia: a cause of ventricular tachycardia in children with apparently normal hearts

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD), a cardiomyopathy with hypokinetic ares limited to the wall of the right ventricle (RV), has been recently described as a cause of recurrent ventricular tachycardia (VT) in young adults with an otherwise normal heart. We reviewed 26 cases of recurrent VT in children and found 10 patients with no clinically recognizable abnormality aside from the dysrhythmia. Three of these 10 patients had ARVD. These three patients were initially seen at 1, 12, and 14 years of age with premature ventricular contractions (PVCs) and/or VT. Sustained VT occurred spontaneously or during stress testing. The PVCs and the VT were of left bundle branch block contour, suggesting RV site of origin. The diagnosis of ARVD was based on wall motion abnormalities of the RV demonstrated angiographically. We suggest that ARVD could be a significantly common cause of VT in children with an apparently normal heart.