• Dylan Therasse, Frederic Sacher, Dominique Babuty, Philippe Mabo, Jacques Mansourati, Florence Kyndt, Richard Redon, Jean Jacques Schott, Julien Barc, Vincent Probst, and Jean-Baptiste Gourraud.
• CHU Nantes, L'institut du thorax, Service de Cardiologie, Nantes F-44000, France.
• Int. J. Cardiol. 2017 Oct 15; 245: 178-180.

AimsIntravenous drug challenge is frequently performed to unmask Brugada syndrome (BrS). However, its true sensitivity has never been assessed. We used the obligate BrS transmitters in families affected by BrS to evaluate the true accuracy of drug challenge.MethodsAll consecutive patients from 2000 to 2014 who underwent drug challenge during familial screening for BrS were included in the study. Obligate BrS transmitters were defined as the presence of a descendant and non-descendant first-degree relative affected by BrS. Two physicians blinded to the clinical and genetic status reviewed the data.ResultsAmong 705 drug challenges performed in 149 families, 50 were performed in obligate transmitters from 42 different families. SCN5A mutations were identified in 20 families. Two obligate transmitters were not carrier of the familial mutation. Based on obligate transmitters, sensitivity was 100% for Ajmaline vs 77% for Flecainide (P=0.002). Based on the presence of the familial SCN5A mutation in all family relatives, sensitivity and specificity of sodium channel blocker challenge were respectively 78% (95/122) and 46% (68/148). During a median follow-up of 91 (26-136) months, 2 ventricular fibrillations occurred in obligate transmitters.ConclusionWe demonstrated that Ajmaline challenge presents an excellent sensitivity that may rule out the diagnosis of BrS when negative. Conversely, a negative Flecainide challenge may not prevent from Brs inheritance and risk of SCD. This may lead to suggest systematic use of Ajmaline during drug challenge.Copyright © 2017 Elsevier B.V. All rights reserved.

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