What is the clinical evidence from this study?

Study design: Case Report. Population: SCN5A p.R222Q-Related Multifocal Ectopic Purkinje-Related Premature Contractions (MEPPC) (n=2). Intervention: Ranolazine. Primary outcome: Suppression of ventricular ectopy and left ventricular function.

What question did this study set out to answer?

The aim is to explore the effectiveness of ranolazine in treating multifocal ectopic Purkinje-related premature contractions caused by the SCN5A p.R222Q variant.

May 22, 2026Journal of Cardiovascular Electrophysiology

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

Q: What does this research mean for the field?

Ranolazine effectively suppresses ventricular ectopy and preserves left ventricular function in patients with SCN5A-related multifocal ectopic Purkinje-related premature contractions (MEPPC). Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

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Key result

Ranolazine suppresses ventricular ectopy and preserves LV function in two siblings with an SCN5A variant.

Why the study?

Gain-of-function variants in SCN5A cause multifocal ectopic Purkinje-related premature contractions (MEPPC) and PVC-induced cardiomyopathy requiring pharmacological therapy, as catheter ablation is ineffective for multifocal ectopy.

Does ranolazine suppress ventricular ectopy and improve left ventricular function in patients with SCN5A p.Arg222Gln-related MEPPC?

Key Points

The aim is to explore the effectiveness of ranolazine in treating multifocal ectopic Purkinje-related premature contractions caused by the SCN5A p.R222Q variant.
Case study of two siblings with the pathogenic SCN5A p.Arg222Gln variant.
Treatment with ranolazine to assess its effect on ventricular ectopy and left ventricular function.
Evaluation of amiodarone withdrawal after ranolazine treatment.
Ranolazine markedly suppressed ventricular ectopy in both siblings.
Left ventricular function improved or was preserved following treatment.
Amiodarone withdrawal was possible due to the successful management of ectopy with ranolazine.

Study Design

Type

Case Report (n=2)

Structured PICO

Does ranolazine suppress ventricular ectopy and improve left ventricular function in patients with SCN5A p.Arg222Gln-related MEPPC?

Population

2 siblings with the pathogenic SCN5A p.Arg222Gln variant causing multifocal ectopic Purkinje-related premature contractions (MEPPC)

Intervention

Ranolazine

Outcome

Suppression of ventricular ectopysurrogate

Ranolazine may represent a useful mechanism-guided pharmacological option for SCN5A-related multifocal ectopic Purkinje-related premature contractions.

Discussion

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Implication

Ranolazine may suppress ectopy in SCN5A-MEPPC; leaves open efficacy and safety in larger cohorts.

Cite This Study

Fonseca et al. (2026) conducted a case report in SCN5A p.R222Q-Related Multifocal Ectopic Purkinje-Related Premature Contractions (MEPPC) (n=2). Ranolazine was evaluated on Suppression of ventricular ectopy and left ventricular function. Ranolazine markedly suppressed ventricular ectopy, improved or preserved left ventricular function, and enabled amiodarone withdrawal in two siblings with the SCN5A p.Arg222Gln variant.

synapsesocial.com/papers/6a0ff43fd674f7c03778d7eb https://doi.org/10.1111/jce.70371

Topics

Cardiomyopathy

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

Key Points

Study Design

Structured PICO

Discussion

Implication

Cite This Study

Topics

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

Key Points

Study Design

Structured PICO

Discussion

Implication

Cite This Study

Also Consider

Topics

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