Effect of Flecainide on Multifocal Ectopic Purkinje-Related Premature Contractions in an R814W SCN5A Carrier

Hisham Ahamed; Arun Gopi

doi:10.1016/j.jaccas.2024.102223

Effect of Flecainide on Multifocal Ectopic Purkinje-Related Premature Contractions in an R814W SCN5A Carrier

JACC Case Rep. 2024 Jan 23;29(5):102223. doi: 10.1016/j.jaccas.2024.102223. eCollection 2024 Mar 6.

Authors

Hisham Ahamed¹, Arun Gopi²

Affiliations

¹ Amrita Institute of Medical Sciences and Research Centre, Edappally, Ernakulam, India.
² Metromed International Cardiac Centre, Calicut, India.

Abstract

Multifocal ectopic Purkinje-related premature contraction (MEPPC) is an autosomal dominant SCN5A channelopathy characterized by frequent multiform premature ventricular contractions originating from the His-Purkinje system. We present a patient with an MEPPC phenotype whose genetic testing identified a pathogenic SCN5A (HGNC:10593) variant amenable to precision antiarrhythmic therapy with flecainide.

Keywords: SCN5A channelopathy; arrhythmogenic cardiomyopathy; cardiomyopathy; dilated cardiomyopathy; mutation.

Publication types

Case Reports