Outcomes of defibrillator therapy in catecholaminergic polymorphic ventricular tachycardia

Rosés‐Noguer, Ferran; Jarman, Julian W.E.; Clague, Jonathan R.; Till, Jan

doi:10.1016/j.hrthm.2013.10.027

Cited by 108 publications

(56 citation statements)

References 30 publications

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“…Careful ICD programming is necessary in CPVT because the effectiveness of appropriate shocks critically depends on the arrhythmia mechanism, usually being effective only when the treated rhythm is ventricular fibrillation. 31,32 Thus, CPVT patients with arrhythmic events despite β-blockers are in dire need of an effective adjunct therapy. The present data conclusively indicate that LCSD represents a viable and effective answer to this predicament.…”

Section: Discussionmentioning

confidence: 99%

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia

et al. 2015

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Background-Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) are used but often cause multiple shocks. Preliminary results with flecainide appear encouraging.We proposed left cardiac sympathetic denervation (LCSD) as useful additional therapy, but evidence remains anecdotal. Methods and Results-We report 63 patients with CPVT who underwent LCSD as secondary (n=54) or primary (n=9) prevention. The median post-LCSD follow-up was 37 months. The 9 asymptomatic patients remained free of major cardiac events. Of the 54 patients with prior major cardiac events either on (n=38) or off (n=16) optimal medical therapy, 13 (24%) had at least 1 recurrence: 0 patients had an aborted cardiac arrest, 2 patients had syncope only, 10 patients had ≥1 appropriate ICD discharges, and 1 patient died suddenly. The 1-and 2-year cumulative event-free survival rates were 87% and 81%. The percentage of patients with major cardiac events despite optimal medical therapy (n=38) was reduced from 100% to 32% (P<0.001) after LCSD, and among 29 patients with a presurgical ICD, the rate of shocks dropped by 93% from 3.6 to 0.6 shocks per person per year (P<0.001). Patients with an incomplete LCSD (n=7) were more likely to experience major cardiac events after LCSD (71% versus 17%; P<0.01) than those with a complete LCSD. Conclusions-LCSD is an effective antifibrillatory intervention for patients with CPVT. Whenever syncope occurs despite optimal medical therapy, LCSD could be considered the next step rather than an ICD and could complement ICDs in patients with recurrent shocks.

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Section: Discussionmentioning

confidence: 99%

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia

et al. 2015

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“…Wszczepienie ICD należy rozważyć również u pacjentów z CPVT, którzy nie odpowiadają na beta-adrenolityki i flekainid [14]. Implantowany kardiowerter-defibrylator należy programować z długimi opóźnieniami przed wyładowaniem, ponieważ bolesne wyładowanie może zwiększać napięcie współczulne i wyzwalać kolejne zaburzenia rytmu, co prowadzi do złośliwego cyklu wyładowań ICD, a nawet zgonu [466].…”

Section: Stratyfikacja Ryzyka I Postępowanieunclassified

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Priori

Blomström‐Lundqvist²,

Mazzanti³

et al. 2015

Kardiol Pol

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“…Unsuccessful shocks were frequently observed in cases of polymorphic VT or bidirectional VT, whereas shocks administered for VF were often successful. 65, 66 Patients with inherited cardiac arrhythmia disorders are exposed to the potential harm of ICD therapy for a long period of time and are therefore at higher risk. A recent meta-analysis demonstrated that inappropriate ICD shocks are significantly more often observed in patients with CPVT (36% vs. 20%; P=0.04) when compared with other inherited arrhythmia syndromes and most often occurred because of supraventricular tachycardia.…”

Section: β-Blockersmentioning

confidence: 99%

Catecholaminergic Polymorphic Ventricular Tachycardia

Lieve

Werf

Wilde

2016

Circ J

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac arrhythmia disorder that is characterized by emotion-and exercise-induced polymorphic ventricular arrhythmias and may lead to sudden cardiac death (SCD). CPVT plays an important role in SCD in the young and therefore recognition and adequate treatment of the disease are of vital importance. In the past years tremendous improvements have been made in the diagnostic methods and treatment of the disease. In this review, we summarize the clinical characteristics, genetics, and diagnostic and therapeutic strategies of CPVT and describe the most recent advances and some of the current challenges. (Circ J 2016; 80: 1285 -1291

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Outcomes of defibrillator therapy in catecholaminergic polymorphic ventricular tachycardia

Cited by 108 publications

References 30 publications

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia

Clinical Management of Catecholaminergic Polymorphic Ventricular Tachycardia

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Catecholaminergic Polymorphic Ventricular Tachycardia

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