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Presentation transcript:

Volume 11, Issue 1, Pages 58-66 (January 2014) Outcomes of defibrillator therapy in catecholaminergic polymorphic ventricular tachycardia Ferran Roses-Noguer, MD, Julian W.E. Jarman, MRCP, MD(Res), Jonathan R. Clague, MD, FRCP, Jan Till, MD Heart Rhythm Volume 11, Issue 1, Pages 58-66 (January 2014) DOI: 10.1016/j.hrthm.2013.10.027 Copyright © 2014 Heart Rhythm Society Terms and Conditions

Figure 1 Exercise-induced ventricular arrhythmias in CPVT. This 24-hour ECG Holter recording from a 15-year-old boy with calsequestrin-positive CPVT well illustrates the challenges faced in programming ICDs in such patients. A series of ventricular arrhythmias is observed as ventricular ectopic beats transition to sustained polymorphic ventricular arrhythmias before settling again to sinus tachycardia. CPVT = catecholaminergic polymorphic ventricular tachycardia; ECG = electrocardiogram; ICD = implantable cardioverter-defibrillator. Heart Rhythm 2014 11, 58-66DOI: (10.1016/j.hrthm.2013.10.027) Copyright © 2014 Heart Rhythm Society Terms and Conditions

Figure 2 Transition from triggered to reentrant arrhythmia. This 12-lead ECG from a 9-year-old boy with ryanodine-positive CPVT shows a transition from triggered bidirectional ventricular tachycardia followed by brief polymorphic ventricular tachycardia to reentrant ventricular fibrillation. CPVT = catecholaminergic polymorphic ventricular tachycardia; ECG = electrocardiogram. Heart Rhythm 2014 11, 58-66DOI: (10.1016/j.hrthm.2013.10.027) Copyright © 2014 Heart Rhythm Society Terms and Conditions

Figure 4 Ineffective and effective ICD shocks. ICD electrograms showing shock therapy for triggered (A) and reentrant (B) arrhythmias. A: Triggered polymorphic and bidirectional VT is unaffected by a 31-J shock. Electrogram channels from top to bottom are ventricular bipolar and ventricular unipolar. B: Ventricular fibrillation is successfully terminated by a 41-J shock. Electrogram channels from top to bottom are atrial bipolar, ventricular bipolar, and ventricular unipolar. ICD = implantable cardioverter-defibrillator; VT = ventricular tachycardia. Heart Rhythm 2014 11, 58-66DOI: (10.1016/j.hrthm.2013.10.027) Copyright © 2014 Heart Rhythm Society Terms and Conditions

Figure 3 Time to first appropriate shock (years). Kaplan-Meier curve presenting time from first ICD implantation to first appropriate shock for ventricular tachyarrhythmia among all patients with CPVT who had undergone ICD implantation (n = 13). CPVT = catecholaminergic polymorphic ventricular tachycardia; ICD = implantable cardioverter-defibrillator. Heart Rhythm 2014 11, 58-66DOI: (10.1016/j.hrthm.2013.10.027) Copyright © 2014 Heart Rhythm Society Terms and Conditions