OBJECTIVES: This study evaluated the efficacy and safety of flecainide in addition to conventional drug therapy in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). BACKGROUND: CPVT is an inherited arrhythmia syndrome caused by gene mutations that destabilize cardiac ryanodine receptor Ca(2+) release channels. Sudden cardiac death is incompletely prevented by conventional drug therapy with β-blockers with or without Ca(2+) channel blockers. The antiarrhythmic agent flecainide directly targets the molecular defect in CPVT by inhibiting premature Ca(2+) release and triggered beats in vitro. METHODS: We collected data from every consecutive genotype-positive CPVT patient started on flecainide at 8 international centers before December 2009. The primary outcome measure was the reduction of ventricular arrhythmias during exercise testing. RESULTS: Thirty-three patients received flecainide because of exercise-induced ventricular arrhythmias despite conventional (for different reasons, not always optimal) therapy (median age 25 years; range 7 to 68 years; 73% female). Exercise tests comparing flecainide in addition to conventional therapy with conventional therapy alone were available for 29 patients. Twenty-two patients (76%) had either partial (n = 8) or complete (n = 14) suppression of exercise-induced ventricular arrhythmias with flecainide (p < 0.001). No patient experienced worsening of exercise-induced ventricular arrhythmias. The median daily flecainide dose in responders was 150 mg (range 100 to 300 mg). During a median follow-up of 20 months (range 12 to 40 months), 1 patient experienced implantable cardioverter-defibrillator shocks for polymorphic ventricular arrhythmias, which were associated with a low serum flecainide level. In 1 patient, flecainide successfully suppressed exercise-induced ventricular arrhythmias for 29 years. CONCLUSIONS: Flecainide reduced exercise-induced ventricular arrhythmias in patients with CPVT not controlled by conventional drug therapy.
OBJECTIVES: This study evaluated the efficacy and safety of flecainide in addition to conventional drug therapy in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). BACKGROUND: CPVT is an inherited arrhythmia syndrome caused by gene mutations that destabilize cardiac ryanodine receptor Ca(2+) release channels. Sudden cardiac death is incompletely prevented by conventional drug therapy with β-blockers with or without Ca(2+) channel blockers. The antiarrhythmic agent flecainide directly targets the molecular defect in CPVT by inhibiting premature Ca(2+) release and triggered beats in vitro. METHODS: We collected data from every consecutive genotype-positive CPVT patient started on flecainide at 8 international centers before December 2009. The primary outcome measure was the reduction of ventricular arrhythmias during exercise testing. RESULTS: Thirty-three patients received flecainide because of exercise-induced ventricular arrhythmias despite conventional (for different reasons, not always optimal) therapy (median age 25 years; range 7 to 68 years; 73% female). Exercise tests comparing flecainide in addition to conventional therapy with conventional therapy alone were available for 29 patients. Twenty-two patients (76%) had either partial (n = 8) or complete (n = 14) suppression of exercise-induced ventricular arrhythmias with flecainide (p < 0.001). No patient experienced worsening of exercise-induced ventricular arrhythmias. The median daily flecainide dose in responders was 150 mg (range 100 to 300 mg). During a median follow-up of 20 months (range 12 to 40 months), 1 patient experienced implantable cardioverter-defibrillator shocks for polymorphic ventricular arrhythmias, which were associated with a low serum flecainide level. In 1 patient, flecainide successfully suppressed exercise-induced ventricular arrhythmias for 29 years. CONCLUSIONS:Flecainide reduced exercise-induced ventricular arrhythmias in patients with CPVT not controlled by conventional drug therapy.
Authors: H Swan; K Piippo; M Viitasalo; P Heikkilä; T Paavonen; K Kainulainen; J Kere; P Keto; K Kontula; L Toivonen Journal: J Am Coll Cardiol Date: 1999-12 Impact factor: 24.094
Authors: Fredrick A Hilliard; Derek S Steele; Derek Laver; Zhaokang Yang; Sylvain J Le Marchand; Nagesh Chopra; David W Piston; Sabine Huke; Björn C Knollmann Journal: J Mol Cell Cardiol Date: 2009-10-14 Impact factor: 5.000
Authors: Argelia Medeiros-Domingo; Zahurul A Bhuiyan; David J Tester; Nynke Hofman; Hennie Bikker; J Peter van Tintelen; Marcel M A M Mannens; Arthur A M Wilde; Michael J Ackerman Journal: J Am Coll Cardiol Date: 2009-11-24 Impact factor: 24.094
Authors: Hiroshi Watanabe; Nagesh Chopra; Derek Laver; Hyun Seok Hwang; Sean S Davies; Daniel E Roach; Henry J Duff; Dan M Roden; Arthur A M Wilde; Björn C Knollmann Journal: Nat Med Date: 2009-03-29 Impact factor: 53.440
Authors: Kristina Hermann Haugaa; Ida Skrinde Leren; Knut Erik Berge; Jørn Bathen; Jan Pål Loennechen; Ole-Gunnar Anfinsen; Andreas Früh; Thor Edvardsen; Erik Kongsgård; Trond P Leren; Jan P Amlie Journal: Europace Date: 2010-01-26 Impact factor: 5.214
Authors: Sonia Franciosi; Thomas M Roston; Frances K G Perry; Bjorn C Knollmann; Prince J Kannankeril; Shubhayan Sanatani Journal: J Cardiovasc Electrophysiol Date: 2019-07-11
Authors: Michael Arad; Michael Glikson; Dalia El-Ani; Lorenzo Monserrat-Inglesias Journal: Ann Noninvasive Electrocardiol Date: 2012-10 Impact factor: 1.468