PO-03-171 MULTIFOCAL ECTOPIC PREMATURE PURKINJE-RELATED COMPLEXES SYNDROME IN CHILDREN

Multifocal ectopic premature Purkinje-related complexes syndrome is a rare form of arrhythmia involving the entire His-Purkinje system (HPS), characterized by frequent polymorphic ventricular contractions (PVCs) relatively narrow QRS, which could be arising from HPS and associated with high probability dilated cardiomyopathy. We presented clinical courses 6 pediatric patients multifocal syndrome. The charts all diagnosis in our institution were reviewed. was diagnosed 2 newborn infants 4 adolescents unrelated families. Two them medical refractory incessant tachycardia underwent multiple catheter ablations complete resolution one. Both had pacemaker therapy for ablation induced heart block. Four medically treated successfully: One propranolol; one ivabradine; flecainide. Pathogenic SCN5A mutation detected should considered children PVCs QRS. Ivabradine or flecainide can reduce burden. In severe cases, provide cure albeit risk