• Frédéric Sacher, Vincent Probst, Philippe Maury, Dominique Babuty, Jacques Mansourati, Yuki Komatsu, Christelle Marquie, Antonio Rosa, Abou Diallo, Romain Cassagneau, Claire Loizeau, Raphael Martins, Michael E Field, Nicolas Derval, Shinsuke Miyazaki, Arnaud Denis, Akihiko Nogami, Philippe Ritter, Jean-Baptiste Gourraud, Sylvain Ploux, Anne Rollin, Adlane Zemmoura, Dominique Lamaison, Pierre Bordachar, Bertrand Pierre, Pierre Jaïs, Jean-Luc Pasquié, Mélèze Hocini, François Legal, Pascal Defaye, Serge Boveda, Yoshito Iesaka, Philippe Mabo, and Michel Haïssaguerre.
• Hôpital Cardiologique du Haut-Lévêque, CHU de Bordeaux, L'Institut de Rythmologie et de Modelisation Cardiaque, INSERM 1045, Bordeaux, France (F.S., A.D., C.L., M.E.F., N.D., A.D., P.R., S.P., A.Z., P.B., P.J., M.H., M.H.); Institut du Thorax, CHU de Nantes, Nantes, France (V.P., J.-B.G.); CHU de Toulouse, Toulouse, France (P. Maury, A. Rollin); CHU de Tours, Tours, France (D.B., B.P.); CHU de Brest, Brest, France (J.M., S.M.); Tsuchiura Kyodo Hospital, Tsuchiura, Japan (Y.K., Y.I.); CHU de Lille, Lille, France (C.M.); Clinique Pasteur, Toulouse, France (A. Rosa, S.B.); CHU de Grenoble, Grenoble, France (R.C., P.D.); CHU de Rennes, Rennes, France (R.M., P. Mabo); Yokohama Rosai Hospital, Yokohama, Japan (A.N.); CHU de Clermont-Ferrand, Clermont-Ferrand, France (D.L.); CHU de Montpellier, Montpellier, France (J.-L.P.); and CHU de Poitiers, Poiters, France (F.L.).
• Circulation. 2013 Oct 15; 128 (16): 1739-47.

BackgroundImplantable cardioverter-defibrillator indications in Brugada syndrome remain controversial, especially in asymptomatic patients. Previous outcome data are limited by relatively small numbers of patients or short follow-up durations. We report the outcome of patients with Brugada syndrome implanted with an implantable cardioverter-defibrillator in a large multicenter registry.Methods And ResultsA total of 378 patients (310 male; age, 46±13 years) with a type 1 Brugada ECG pattern implanted with an implantable cardioverter-defibrillator (31 for aborted sudden cardiac arrest, 181 for syncope, and 166 asymptomatic) were included. Fifteen patients (4%) were lost to follow-up. During a mean follow-up of 77±42 months, 7 patients (2%) died (1 as a result of an inappropriate shock), and 46 patients (12%) had appropriate device therapy (5±5 shocks per patient). Appropriate device therapy rates at 10 years were 48% for patients whose implantable cardioverter-defibrillator indication was aborted sudden cardiac arrest, 19% for those whose indication was syncope, and 12% for the patients who were asymptomatic at implantation. At 10 years, rates of inappropriate shock and lead failure were 37% and 29%, respectively. Inappropriate shock occurred in 91 patients (24%; 4±4 shocks per patient) because of lead failure (n=38), supraventricular tachycardia (n=20), T-wave oversensing (n=14), or sinus tachycardia (n=12). Importantly, introduction of remote monitoring, programming a high single ventricular fibrillation zone (>210-220 bpm), and a long detection time were associated with a reduced risk of inappropriate shock.ConclusionsAppropriate therapies are more prevalent in symptomatic Brugada syndrome patients but are not insignificant in asymptomatic patients (1%/y). Optimal implantable cardioverter-defibrillator programming and follow-up dramatically reduce inappropriate shock. However, lead failure remains a major problem in this population.

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