Ferran Roses-Noguer1, Julian W E Jarman1, Jonathan R Clague1, Jan Till2. 1. Department of Cardiology, Royal Brompton Hospital, London, United Kingdom. 2. Department of Cardiology, Royal Brompton Hospital, London, United Kingdom. Electronic address: j.till@rbht.nhs.uk.
Abstract
BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by adrenergically induced ventricular arrhythmias in patients with structurally normal hearts. Initiating triggered arrhythmias, such as bidirectional ventricular tachycardia, often degenerate into reentrant arrhythmias, such as ventricular fibrillation (VF). OBJECTIVE: To determine whether the effectiveness of implantable cardioverter-defibrillator (ICD) shocks is dependent on rhythm type. METHODS: It is a retrospective study of patients with CPVT who had undergone ICD implantation. Thirteen patients received ICDs (median age 15 years; range 9-43 years): 7 of 13 (54%) for cardiac arrest and 6 of 13 (46%) for syncope despite drug therapy. The median follow-up duration was 4.0 years (range 1.7-19.9 years). Nineteen reinterventions occurred, excluding generator replacements. Ten patients received 96 shocks (median 4 shocks; range 1-30 shocks). Eighty-seven shock electrograms were reviewed. Sixty-three (72%) shocks were appropriate, and 24 (28%) were inappropriate (T-wave oversensing 7 [29%], supraventricular arrhythmia 16 [67%], after self-terminating VF 1 [4%]). RESULTS: Among appropriate shocks, 20 (32%) were effective in terminating sustained arrhythmia and 43 (68%) were ineffective. Shocks delivered to triggered arrhythmias nearly always failed (1 of 40 [3%] effective), while shocks delivered to VF were usually successful (19 of 23 [83%] effective; P < .001). Four patients received 17 appropriate antitachycardia pacing therapies for ventricular tachycardia: only 2 (12%) were effective. No patient died. CONCLUSIONS: The effectiveness of ICD shock therapy in CPVT depends on the mechanism of the rhythm treated. Shocks delivered to initiating triggered arrhythmias nearly always fail, whereas those for subsequent VF are usually effective. ICD programming in these patients is exceptionally challenging.
BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by adrenergically induced ventricular arrhythmias in patients with structurally normal hearts. Initiating triggered arrhythmias, such as bidirectional ventricular tachycardia, often degenerate into reentrant arrhythmias, such as ventricular fibrillation (VF). OBJECTIVE: To determine whether the effectiveness of implantable cardioverter-defibrillator (ICD) shocks is dependent on rhythm type. METHODS: It is a retrospective study of patients with CPVT who had undergone ICD implantation. Thirteen patients received ICDs (median age 15 years; range 9-43 years): 7 of 13 (54%) for cardiac arrest and 6 of 13 (46%) for syncope despite drug therapy. The median follow-up duration was 4.0 years (range 1.7-19.9 years). Nineteen reinterventions occurred, excluding generator replacements. Ten patients received 96 shocks (median 4 shocks; range 1-30 shocks). Eighty-seven shock electrograms were reviewed. Sixty-three (72%) shocks were appropriate, and 24 (28%) were inappropriate (T-wave oversensing 7 [29%], supraventricular arrhythmia 16 [67%], after self-terminating VF 1 [4%]). RESULTS: Among appropriate shocks, 20 (32%) were effective in terminating sustained arrhythmia and 43 (68%) were ineffective. Shocks delivered to triggered arrhythmias nearly always failed (1 of 40 [3%] effective), while shocks delivered to VF were usually successful (19 of 23 [83%] effective; P < .001). Four patients received 17 appropriate antitachycardia pacing therapies for ventricular tachycardia: only 2 (12%) were effective. No patient died. CONCLUSIONS: The effectiveness of ICD shock therapy in CPVT depends on the mechanism of the rhythm treated. Shocks delivered to initiating triggered arrhythmias nearly always fail, whereas those for subsequent VF are usually effective. ICD programming in these patients is exceptionally challenging.
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: 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