BACKGROUND: Calsequestrin-associated catecholaminergic polymorphic ventricular tachycardia (CPVT2) can cause sudden death in young individuals in response to stress. Beta-blockers are the mainstay medical treatment for patients with CPVT2. However, they do not prevent syncope and sudden death in all patients. Flecainide was reported to reduce exercise-induced ventricular arrhythmias (EIVA) in patients with ryanodine receptor-associated CPVT. The role of flecainide in CPVT2 is not known. OBJECTIVE: To summarize our experience in combining flecainide and beta-blockers in high-risk patients with CPVT2. METHODS: All patients with CPVT2 (10 patients) who have high-risk features (syncope, EIVA, or appropriate implantable cardioverter-defibrillator [ICD] shocks) despite beta-blockers with or without calcium channel blockers were treated with a combination of flecainide and beta-blockers. Exercise test was done before and after beginning treatment with flecainide. RESULTS: All patients had EIVA and 4 had appropriate ICD shocks before flecainide treatment. EIVA-included frequent ventricular premature beats and or ventricular tachycardia during the exercise test while on high dose of beta-blockers with or without calcium channel blockers before treatment with flecainide. After combination therapy with flecainide and beta-blockers, EIVA were suppressed completely in all patients. During follow-up of 15.5 ± 10.4 months (range 2-29 months), 8 patients were free of symptoms and free of arrhythmias. Two patients had 1 VT storm episode with recurrent ICD shocks despite repeated normal stress test. CONCLUSIONS: Flecainide can completely prevent ventricular arrhythmia during exercise and partially prevent recurrent ICD shocks in high-risk patients with CPVT2.
BACKGROUND: Calsequestrin-associated catecholaminergic polymorphic ventricular tachycardia (CPVT2) can cause sudden death in young individuals in response to stress. Beta-blockers are the mainstay medical treatment for patients with CPVT2. However, they do not prevent syncope and sudden death in all patients. Flecainide was reported to reduce exercise-induced ventricular arrhythmias (EIVA) in patients with ryanodine receptor-associated CPVT. The role of flecainide in CPVT2 is not known. OBJECTIVE: To summarize our experience in combining flecainide and beta-blockers in high-risk patients with CPVT2. METHODS: All patients with CPVT2 (10 patients) who have high-risk features (syncope, EIVA, or appropriate implantable cardioverter-defibrillator [ICD] shocks) despite beta-blockers with or without calcium channel blockers were treated with a combination of flecainide and beta-blockers. Exercise test was done before and after beginning treatment with flecainide. RESULTS: All patients had EIVA and 4 had appropriate ICD shocks before flecainide treatment. EIVA-included frequent ventricular premature beats and or ventricular tachycardia during the exercise test while on high dose of beta-blockers with or without calcium channel blockers before treatment with flecainide. After combination therapy with flecainide and beta-blockers, EIVA were suppressed completely in all patients. During follow-up of 15.5 ± 10.4 months (range 2-29 months), 8 patients were free of symptoms and free of arrhythmias. Two patients had 1 VT storm episode with recurrent ICD shocks despite repeated normal stress test. CONCLUSIONS:Flecainide can completely prevent ventricular arrhythmia during exercise and partially prevent recurrent ICD shocks in high-risk patients with CPVT2.
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: Mohamed Y Amarouch; Heikki Swan; Jaakko Leinonen; Annukka Marjamaa; Annukka M Lahtinen; Kimmo Kontula; Lauri Toivonen; Elisabeth Widen; Hugues Abriel Journal: Ann Noninvasive Electrocardiol Date: 2015-10-07 Impact factor: 1.468
Authors: Thomas M Roston; Jeffrey M Vinocur; Kathleen R Maginot; Saira Mohammed; Jack C Salerno; Susan P Etheridge; Mitchell Cohen; Robert M Hamilton; Andreas Pflaumer; Ronald J Kanter; James E Potts; Martin J LaPage; Kathryn K Collins; Roman A Gebauer; Joel D Temple; Anjan S Batra; Christopher Erickson; Maria Miszczak-Knecht; Peter Kubuš; Yaniv Bar-Cohen; Michal Kantoch; Vincent C Thomas; Gabriele Hessling; Chris Anderson; Ming-Lon Young; Michel Cabrera Ortega; Yung R Lau; Christopher L Johnsrude; Anne Fournier; Prince J Kannankeril; Shubhayan Sanatani Journal: Circ Arrhythm Electrophysiol Date: 2015-02-24
Authors: Samantha C Salvage; Karthik H Chandrasekharan; Kamalan Jeevaratnam; Angela F Dulhunty; Andrew J Thompson; Antony P Jackson; Christopher L-H Huang Journal: Br J Pharmacol Date: 2017-05-12 Impact factor: 8.739
Authors: Kirsi Penttinen; Heikki Swan; Sari Vanninen; Jere Paavola; Annukka M Lahtinen; Kimmo Kontula; Katriina Aalto-Setälä Journal: PLoS One Date: 2015-05-08 Impact factor: 3.240