Successful Use of Ranolazine in SCN5A p.R222Q-Related Multifocal Ectopic Purkinje-Related Premature Contractions.

INTRODUCTION

Gain-of-function variants in SCN5A may cause multifocal ectopic Purkinje-related premature contractions (MEPPC), with a high premature ventricular contraction burden that may lead to PVC-induced cardiomyopathy. Pharmacological therapy is usually required, as catheter ablation is ineffective because of the multifocal origin of the ectopy.

METHODS AND

RESULTS

We report two siblings with the pathogenic SCN5A p.Arg222Gln variant. In both, ranolazine markedly suppressed ventricular ectopy, improved or preserved left ventricular function, and enabled amiodarone withdrawal.

CONCLUSION

Ranolazine may represent a useful mechanism-guided option for SCN5A-related MEPPC.