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Article

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

Journal of cardiovascular electrophysiology - 1 Jul 2026

Fonseca Isabella Moreira Gonzalez, França Anna Terra, Carmo André Assis Lopes do, van der Crabben Saskia N, van der Werf Christian, Wilde Arthur A M

Abstract excerpt

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...

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Identifiers

PMID: 42160484
DOI: 10.1111/jce.70371
PMCID: PMC13372430

Genes

SCN5A

Variants

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

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Successful Use of Ranolazine in SCN5A p.R222Q-Related Multifocal Ectopic Purkinje-Related Premature Contractions.PMID 42160484Read ↗

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Multifocal ectopic Purkinje-related premature contractions: a new SCN5A-related cardiac channelopathy.Semantic proximity Read ↗

A novel gain-of-function mutation in SCN5A responsible for multifocal ectopic Purkinje-related premature contractions.Semantic proximity Read ↗

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The G213D variant in Nav1.5 alters sodium current and causes an arrhythmogenic phenotype resulting in a multifocal ectopic Purkinje-related premature contraction phenotype in human-induced pluripotent stem cell-derived cardiomyocytes.Semantic proximity Read ↗

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