ArticleIndian pacing and electrophysiology journal
Think SCN5A: A novel variant causing multifocal Purkinje PVCs and dilated cardiomyopathy.
Article in Indian pacing and electrophysiology journal. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundMultifocal ectopic Purkinje-related premature contractions (MEPPC) represent a rare SCN5A-associated channelopathy characterized by high-burden, multifocal premature ventricular contractions (PVCs) arising from the His-Purkinje system, often leading to reversible left ventricular dysfunction. CASE SUMMARY: We describe a young male with a MEPPC-like phenotype and a novel SCN5A (c.655G > A, p.Arg222Gln) variant presenting with multifocal Purkinje and papillary muscle PVCs and left ventricular systolic dysfunction. Sequential focal ablations reduced the dominant ectopic burden, while adjunctive flecainide therapy further suppressed residual Purkinje activity and improved ventricular function.
conclusionIn young patients with multifocal Purkinje PVCs and unexplained cardiomyopathy, consider SCN5A mutation. Combined ablation and sodium-channel blockade could be considered as a treatment option.
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