BACKGROUND: Conventional therapy with beta-blockers is incompletely effective in preventing arrhythmic events in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). We have previously discovered that flecainide in addition to conventional drug therapy prevents ventricular arrhythmias in patients with genotype-positive CPVT. OBJECTIVE: To study the efficacy of flecainide in patients with genotype-negative CPVT. METHODS: We studied the efficacy of flecainide for reducing ventricular arrhythmias during exercise testing and preventing arrhythmia events during long-term follow-up. RESULTS: Twelve patients with genotype-negative CPVT were treated with flecainide. Conventional therapy failed to control ventricular arrhythmias in all patients. Flecainide was initiated because of significant ventricular arrhythmias (n = 8), syncope (n = 3), or cardiac arrest (n = 1). At the baseline exercise test before flecainide, 6 patients had ventricular tachycardia and 5 patients had bigeminal or frequent ventricular premature beats. Flecainide reduced ventricular arrhythmias at the exercise test in 8 patients compared to conventional therapy, similar to that in patients with genotype-positive CPVT in our previous report. Notably, flecainide completely prevented ventricular arrhythmias in 7 patients. Flecainide was continued in all patients except for one who had ventricular tachycardia at the exercise test on flecainide. During a follow-up of 48±94 months, arrhythmia events (sudden cardiac death and aborted cardiac arrest) associated with noncompliance occurred in 2 patients. Flecainide was not discontinued owing to side effects in any of the patients. CONCLUSIONS: Flecainide was effective in patients with genotype-negative CPVT, suggesting that spontaneous Ca(2+) release from ryanodine channels plays a role in arrhythmia susceptibility, similar to that in patients with genotype-positive CPVT.
BACKGROUND: Conventional therapy with beta-blockers is incompletely effective in preventing arrhythmic events in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). We have previously discovered that flecainide in addition to conventional drug therapy prevents ventricular arrhythmias in patients with genotype-positive CPVT. OBJECTIVE: To study the efficacy of flecainide in patients with genotype-negative CPVT. METHODS: We studied the efficacy of flecainide for reducing ventricular arrhythmias during exercise testing and preventing arrhythmia events during long-term follow-up. RESULTS: Twelve patients with genotype-negative CPVT were treated with flecainide. Conventional therapy failed to control ventricular arrhythmias in all patients. Flecainide was initiated because of significant ventricular arrhythmias (n = 8), syncope (n = 3), or cardiac arrest (n = 1). At the baseline exercise test before flecainide, 6 patients had ventricular tachycardia and 5 patients had bigeminal or frequent ventricular premature beats. Flecainide reduced ventricular arrhythmias at the exercise test in 8 patients compared to conventional therapy, similar to that in patients with genotype-positive CPVT in our previous report. Notably, flecainide completely prevented ventricular arrhythmias in 7 patients. Flecainide was continued in all patients except for one who had ventricular tachycardia at the exercise test on flecainide. During a follow-up of 48±94 months, arrhythmia events (sudden cardiac death and aborted cardiac arrest) associated with noncompliance occurred in 2 patients. Flecainide was not discontinued owing to side effects in any of the patients. CONCLUSIONS:Flecainide was effective in patients with genotype-negative CPVT, suggesting that spontaneous Ca(2+) release from ryanodine channels plays a role in arrhythmia susceptibility, similar to that in patients with genotype-positive CPVT.
Authors: Zahurul A Bhuiyan; Mohamed A Hamdan; Eman T A Shamsi; Alex V Postma; Marcel M A M Mannens; Arthur A M Wilde; Lihadh Al-Gazali Journal: J Cardiovasc Electrophysiol Date: 2007-07-30
Authors: Andrew E Epstein; John P DiMarco; Kenneth A Ellenbogen; N A Mark Estes; Roger A Freedman; Leonard S Gettes; A Marc Gillinov; Gabriel Gregoratos; Stephen C Hammill; David L Hayes; Mark A Hlatky; L Kristin Newby; Richard L Page; Mark H Schoenfeld; Michael J Silka; Lynne Warner Stevenson; Michael O Sweeney; Sidney C Smith; Alice K Jacobs; Cynthia D Adams; Jeffrey L Anderson; Christopher E Buller; Mark A Creager; Steven M Ettinger; David P Faxon; Jonathan L Halperin; Loren F Hiratzka; Sharon A Hunt; Harlan M Krumholz; Frederick G Kushner; Bruce W Lytle; Rick A Nishimura; Joseph P Ornato; Richard L Page; Barbara Riegel; Lynn G Tarkington; Clyde W Yancy Journal: Circulation Date: 2008-05-15 Impact factor: 29.690
Authors: Björn C Knollmann; Nagesh Chopra; Thinn Hlaing; Brandy Akin; Tao Yang; Kristen Ettensohn; Barbara E C Knollmann; Kenneth D Horton; Neil J Weissman; Izabela Holinstat; Wei Zhang; Dan M Roden; Larry R Jones; Clara Franzini-Armstrong; Karl Pfeifer Journal: J Clin Invest Date: 2006-08-24 Impact factor: 14.808
Authors: Rafael Rosso; Jonathan M Kalman; Ori Rogowski; Shmuel Diamant; Amir Birger; Simon Biner; Bernard Belhassen; Sami Viskin Journal: Heart Rhythm Date: 2007-05-24 Impact factor: 6.343
Authors: Arthur A M Wilde; Zahurul A Bhuiyan; Lia Crotti; Mario Facchini; Gaetano M De Ferrari; Thomas Paul; Chiara Ferrandi; Dave R Koolbergen; Attilio Odero; Peter J Schwartz Journal: N Engl J Med Date: 2008-05-08 Impact factor: 91.245
Authors: Marina Cerrone; Sami F Noujaim; Elena G Tolkacheva; Arkadzi Talkachou; Ryan O'Connell; Omer Berenfeld; Justus Anumonwo; Sandeep V Pandit; Karen Vikstrom; Carlo Napolitano; Silvia G Priori; José Jalife Journal: Circ Res Date: 2007-09-13 Impact factor: 17.367
Authors: Matthew M Kalscheur; Ravi Vaidyanathan; Kate M Orland; Sara Abozeid; Nicholas Fabry; Kathleen R Maginot; Craig T January; Jonathan C Makielski; Lee L Eckhardt Journal: Heart Rhythm Date: 2014-02-21 Impact factor: 6.343
Authors: Prince J Kannankeril; Jeremy P Moore; Marina Cerrone; Silvia G Priori; Naomi J Kertesz; Pamela S Ro; Anjan S Batra; Elizabeth S Kaufman; David L Fairbrother; Elizabeth V Saarel; Susan P Etheridge; Ronald J Kanter; Michael P Carboni; Matthew V Dzurik; Darlene Fountain; Heidi Chen; E Wesley Ely; Dan M Roden; Bjorn C Knollmann Journal: JAMA Cardiol Date: 2017-07-01 Impact factor: 14.676
Authors: Andrés R Pérez-Riera; Raimundo Barbosa-Barros; Marianne P C de Rezende Barbosa; Rodrigo Daminello-Raimundo; Augusto A de Lucca; Luiz C de Abreu Journal: Ann Noninvasive Electrocardiol Date: 2017-10-19 Impact factor: 1.468
Authors: Peter J Schwartz; Michael J Ackerman; Charles Antzelevitch; Connie R Bezzina; Martin Borggrefe; Bettina F Cuneo; Arthur A M Wilde Journal: Nat Rev Dis Primers Date: 2020-07-16 Impact factor: 52.329