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Hydroquinidine therapy in Brugada syndrome

Jean-Sylvain Hermida, MD^*,*, Isabelle Denjoy, MD, Jérôme Clerc, MD^*, Fabrice Extramiana, MD, Geneviève Jarry, MD^*, Paul Milliez, MD, Pascale Guicheney, PhD, Stefania Di Fusco, MD, Jean-Luc Rey, MD^*, Bruno Cauchemez, MD and Antoine Leenhardt, MD

^* Amiens-Picardie University Hospital, Amiens, France
Lariboisière University Hospital, Paris, France
Inserm U582, Pitié-Salpêtrière Hospital, Paris, France

View larger version (23K): [in a new window]   Figure 1 Brugada syndrome (BrS) population. Among the BrS cohort followed in both institutions, 80 patients were asymptomatic. From 1999, hydroquinidine therapy was prescribed to asymptomatic patients with BrS and inducible arrhythmia (*) (n = 31). In case of noninducibility, patients were not treated, except in case of multiple familial sudden deaths. In case of drug-induced BrS with a normal baseline electrocardiogram (n = 12), the electrophysiologic (EP) study was not performed. The study population also includes four BrS patients () who had multiple implantable cardioverter-defibrillator (ICD) shocks. SD = sudden death; VF = ventricular fibrillation.

View larger version (23K): [in a new window]   Figure 2 Electrophysiologically guided therapy. After a trial period with hydroquinidine (HQ), BrS patients underwent EP study (EPS). Of 31 asymptomatic patients with BrS and inducible arrhythmia, 29 underwent an EP study with HQ. The rate of ventricular tachycardia (VT)/ventricular fibrillation (VF) noninducibility was 76% (22 of 29). Abbreviations as in Figure 1.

View larger version (72K): [in a new window]   Figure 3 (A) Major QTc interval prolongation observed in a patient who had syncope only one month after the beginning of HQ. The SCN5A mutation was not found in this patient. Measurement of the QT interval includes the inverted T wave observed after the downsloping ST-segment elevation in lead V2. (B) Monomorphic runs of VT were recorded in the emergency room, but in only one lead. No recurrence of syncope was observed after withdrawal of HQ during a follow-up of one year. Syncope may be attributed to a drug inefficacy or to a proarrhythmic effect of HQ. The argument for drug failure is the absence of typical pause-dependent torsade de pointes and of a long coupling interval. The argument for a proarrhythmic event is major QTc interval prolongation. Abbreviations as in Figure 1.

View larger version (24K): [in a new window]   Figure 4 Effect of HQ in one of the four patients with BrS and multiple ICD shocks. Control = numerous salvoes of VT; HQ = dramatic reduction of ventricular arrhythmia after two days of treatment. Abbreviations as in Figure 1.