Nathalie Gaborit, Thomas Wichter, Andras Varro, Viktoria Szuts, Guillaume Lamirault, Lars Eckardt, Matthias Paul, Günter Breithardt, Eric Schulze-Bahr, Denis Escande, Stanley Nattel, Sophie Demolombe
AIMS: Brugada syndrome is an inherited sudden-death arrhythmia syndrome. Na(+)-current dysfunction is central, but mutations in the SCN5A gene (encoding the cardiac Na(+)-channel Nav1.5) are present in only 20% of probands. This study addressed the possibility that Brugada patients display specific expression patterns for ion-channels regulating cardiac conduction, excitability, and repolarization. METHODS AND RESULTS: Transcriptional profiling was performed on right-ventricular endomyocardial biopsies from 10 unrelated Brugada probands, 11 non-diseased organ-donors, seven heart-transplant recipients, 10 with arrhythmogenic right-ventricular cardiomyopathy, and nine with idiopathic right-ventricular outflow-tract tachycardia...
February 2009: European Heart Journal