Key result
Flecainide reduces PVC burden by ~28 percentage points and normalizes LVEF in SCN5A-variant MEPPC syndrome.
Why the study?
Does flecainide improve PVC burden and left ventricular function in a patient with MEPPC syndrome and an SCN5A R814W mutation?
Case Report (n=1)
Does flecainide improve PVC burden and left ventricular function in a patient with MEPPC syndrome and an SCN5A R814W mutation?
Absolute Event Rate: 3% vs 31%
Flecainide effectively reduced PVC burden and reversed dilated cardiomyopathy in a patient with MEPPC syndrome caused by a pathogenic SCN5A R814W gain-of-function variant.
Flecainide may support targeted use in SCN5A-MEPPC; leaves open efficacy and safety in larger prospective cohorts.
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.
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Ahamed et al. (2024) conducted a case report in Multifocal ectopic Purkinje-related premature contraction (MEPPC) and dilated cardiomyopathy (n=1). Flecainide vs. Baseline (pretreatment) was evaluated on Premature ventricular contraction (PVC) burden (%). Flecainide therapy reduced PVC burden from 31% to 3% and improved left ventricular ejection fraction from 35% to 55% in a patient with MEPPC syndrome and a pathogenic SCN5A R814W variant.
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