Multifocal ectopic premature Purkinje-related complexes syndrome in children

Key Teaching Points•Multifocal ectopic premature Purkinje-related complexes (MEPPC) are characterized by frequent polymorphic premature ventricular contractions (PVCs) of short coupling interval, sharp initial QRS deflection, relatively narrow QRS duration (Purkinje system hyperexcitability), and a family history, and is associated with dilated cardiomyopathy. MEPPC should be considered in young patients with frequent multifocal PVCs with relatively narrow QRS.•Mutations in SCN5A genes were shown to be associated with MEPPC. However, presence of an SCN5A variant is not required for the diagnosis.•In the literature, quinidine, hydroquinidine, amiodarone, and flecainide all have been reported as treating the PVCs with success. Ivabradine, a relatively new drug, can be useful in some MEPPC patients.•In severe cases with ventricular tachycardias, extensive catheter ablations may be required to achieve freedom from arrhythmias. Owing to the proximity of lesions to the His bundle, ablation-induced heart block may be inevitable. Therefore, medications should first be tried for rhythm control, which may result in normalization of the LV function in at least some patients. •Multifocal ectopic premature Purkinje-related complexes (MEPPC) are characterized by frequent polymorphic premature ventricular contractions (PVCs) of short coupling interval, sharp initial QRS deflection, relatively narrow QRS duration (Purkinje system hyperexcitability), and a family history, and is associated with dilated cardiomyopathy. MEPPC should be considered in young patients with frequent multifocal PVCs with relatively narrow QRS.•Mutations in SCN5A genes were shown to be associated with MEPPC. However, presence of an SCN5A variant is not required for the diagnosis.•In the literature, quinidine, hydroquinidine, amiodarone, and flecainide all have been reported as treating the PVCs with success. Ivabradine, a relatively new drug, can be useful in some MEPPC patients.•In severe cases with ventricular tachycardias, extensive catheter ablations may be required to achieve freedom from arrhythmias. Owing to the proximity of lesions to the His bundle, ablation-induced heart block may be inevitable. Therefore, medications should first be tried for rhythm control, which may result in normalization of the LV function in at least some patients.