Recognizing Pathogenic PVCs in Children: A Case of Multifocal Ectopic Purkinje-Related Premature Contractions

Premature ventricular contractions (PVCs) are a common and benign arrhythmia in children, but they can infrequently be a sign of an underlying cardiac issue that may predispose patients to heart failure and/or sudden cardiac death (SCD). A 13-year-old, otherwise, healthy female presented for a routine annual evaluation and was found to have an irregular heartbeat. Initial ECGs showed frequent multiform PVCs, premature junctional contractions (PJCs), and short nonsustained runs of ventricular tachycardia (NSVT). PVCs were characterized by sharp initial QRS deflection and relatively narrow QRS duration pointing toward an origin from the Purkinje system. Inpatient 24-h Holter monitor showed frequent multiform PVCs and short runs of NSVT with a 48% burden. Echocardiogram showed a structurally normal heart but moderately depressed left ventricular function. Family history was positive for several individuals on the paternal side who experienced SCD and cardiomyopathy. Specifically, her paternal aunt had symptomatic multiform PVCs which have been refractory to multiple antiarrhythmic medications and catheter ablations. Her aunt's genetic testing was significant for a variant of uncertain significance (VUS) in SCN5A. Multifocal Ectopic Purkinje-related Premature Contractions (MEPPC) was suspected and flecainide was started. Within a few days, she had resolution of ventricular arrhythmias and repeat echocardiogram showed improvement in function. This case provides general practitioners with an illustrative example of pathogenic PVCs in contrast to benign findings, aiding in early recognition and appropriate referral. It also represents an important example of MEPPC in a pediatric patient responding well to flecainide, which is of particular relevance to cardiologists and electrophysiologists.