Successful Use of Quinidine in Treatment of Electrical Storm in Brugada Syndrome

Abstract

We report an adolescent with a malignant form of Brugada syndrome who presented with 15 episodes of ventricular fibrillation (VF) over 10 days, shortly after implantation of an implantable cardioverter defibrillator. Oral quinidine bisulphate at a dose of 1000 mg/day successfully suppressed the electrical storm and recurrence of VF over 18-month follow-up. It also normalized the ST-segment elevation in his right precordial leads, suppressed all ambient unifocal ventricular extrasystoles and induction of VF on programmed electrical stimulation. This case suggests that quinidine, by virtue of its blocking action on Ito, may be useful as adjunctive therapy in Brugada syndrome.

Successful Radiofrequency Catheter Ablation for Electrical Storm of Ventricular Fibrillation in a Patient With Brugada Syndrome

“…13 The effectiveness of quinidine in treating ES in patients with BS has been indicated in some reports. 2,3 Cilostazol, an oral phosphodiesterase type III inhibitor that increases ICa, is also reported to have suppressed the frequently occurring VF episodes in a patient with BS 4 In the present case, ISP successfully normalized the STsegment and suppressed the occurrence of VF. However, quinidine and cilostazol were ineffective in suppressing VF.…”

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

“…It is of utmost importance to try and understand the substrate of incessant arrhythmias, because if a diagnosis is established, a targeted treatment may be possible. For example, in Brugada syndrome, quinidine or isoproterenol may terminate incessant arrhythmias (139,395). In acute ischemia, intravenous amiodarone seems more effective than other antiarrhythmic drugs (396).…”

Therapy for the Brugada Syndrome

“…The authors concluded that quinidine effectively suppresses VF induction as well as spontaneous arrhythmias in patients with Brugada syndrome and may be useful as an adjunct to ICD therapy or as an alternative to ICD in cases in which an ICD is refused, unaffordable, or not feasible for any reason. These results are consistent with those reported the same group in prior years (Belhassen et al 1999(Belhassen et al 2002 and more recently by other investigators (Hermida et al 2004;Mok et al 2004). The data highlight the need for randomized clinical trials to assess the effectiveness of quinidine, preferably in patients with frequent events who have already received an ICD.…”

“…Isoproterenol, sometimes in combination with quinidine, has been shown to be effective in normalizing ST segment elevation in patients with the Brugada syndrome and in controling electrical storms, particularly in children (Alings et al 2001;Shimizu et al 2000b;Suzuki et al 2000;Tanaka et al 2001;Belhassen et al 2002;Mok et al 2004).…”

“…a Control, BCL 800 ms. b Terfenadine (5 μM) induces ST segment elevation as a result of heterogeneous loss of the epicardial action potential dome, leading to phase 2 reentry3 which triggers a closely coupled extrasystole (BCL = 800 ms). c Addition of AVEO 118 (7 μM) prevents loss of the epicardial action potential dome and phase 2 reentry-induced arrhythmias (BCL = 800 ms) Table 2 Device nad pharmacologic apaproach to therapy of the Brugada syndrome Devices and Ablation ICD (Brugada et al 2000a) ?Ablation or Cryosurgery (Haissaguerre et l. 2003) ?Pacemaker (vn den Berg et al 2001) Pharmcaologic Approach to Therapy Ineffective Amiodarone β-Blockers (Brugadaa et al 1998) Class iC antiarrhythmics Flecainide (Shimizu et al 2000a) Propafenone (Matana et al 2000) ?Disopyramide (Chinushi et al 1997) Class IA aaantiarrhythmics Procainaamide (Brugada et al 2000c) Effective for treatment of electrical storms β-Adrenergic agonists-isoproterenol (Miyazaki et al 1996;Shihmizu et al 2000b) Phosphodiesterase III inhibitors-cilostazol (Tsuchiya et al 2002) Effective general therapy Class IA antiarrythmics Quinidine (Belhassen and Viskin 2004;Alings et al 2001;Belhassen et al 1999Belhassen et al , 2002Yan and Antzelevitch 1999;Hermida et al 2004;Mok et al 2004) Experimental therapy I to blockers-cardioselective and ion channel specific Quinidine (Yan and Antzelevitch 1999) 4-Aaminopyridine (Yan and Antzelevitch 1999) Tedisamil (Fish et al 2004b) AVE0118 (Fish et al 2004a) …”