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- W4377014808 abstract "Multifocal ectopic premature Purkinje-related complexes syndrome is a rare form of arrhythmia involving the entire His-Purkinje system (HPS), characterized by frequent polymorphic premature ventricular contractions (PVCs) of relatively narrow QRS, which could be arising from the entire HPS and is associated with a high probability of dilated cardiomyopathy. We presented the clinical courses of 6 pediatric patients with multifocal ectopic premature Purkinje-related complexes syndrome. The charts of all pediatric patients with the diagnosis of multifocal ectopic premature Purkinje-related complexes syndrome in our institution were reviewed. Multifocal ectopic premature Purkinje-related complexes syndrome was diagnosed in 2 newborn infants and 4 adolescents in 4 unrelated families. Two 2 of them with medical refractory incessant tachycardia underwent multiple catheter ablations with complete resolution of arrhythmia in one. Both patients had pacemaker therapy for ablation induced complete heart block. Four patients were medically treated successfully: One with propranolol; one with ivabradine; and 2 with flecainide. Pathogenic SCN5A mutation was detected in 2 families. Multifocal ectopic premature Purkinje-related complexes syndrome should be considered in infants and children with frequent multifocal PVCs with relatively narrow QRS. Ivabradine or flecainide can reduce the arrhythmia burden. In severe cases, catheter ablation can provide cure albeit with high risk of ablation induced heart block." @default.
- W4377014808 created "2023-05-19" @default.
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- W4377014808 date "2023-05-01" @default.
- W4377014808 modified "2023-10-01" @default.
- W4377014808 title "PO-03-171 MULTIFOCAL ECTOPIC PREMATURE PURKINJE-RELATED COMPLEXES SYNDROME IN CHILDREN" @default.
- W4377014808 doi "https://doi.org/10.1016/j.hrthm.2023.03.967" @default.
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