Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review

Roston, Thomas M.; Jones, Karolina; Hawkins, Nathaniel M.; Bos, J. Martijn; Schwartz, Peter J.; Perry, Frances; Ackerman, Michael J.; Laksman, Zachary; Kaul, Padma; Lieve, Krystien V.V.; Atallah, Joseph; Krahn, Andrew D.; Sanatani, Shubhayan

doi:10.1016/j.hrthm.2018.06.046

Cited by 90 publications

(63 citation statements)

References 25 publications

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“…All ventricular ectopy extinguished as the heart rate fell below 100 bpm (E) to avoid implantable cardiac defibrillator (ICD) placement which is associated with a high burden of complications. 10 This is in accordance with patients falling into the Class I category of the 2013 Heart Rhythm Society Consensus Statement on CPVT. 11…”

Section: Discussionsupporting

confidence: 79%

Flipping syncope: The case of an adolescent athlete with syncopal episodes ultimately diagnosed with catecholaminergic polymorphic ventricular tachycardia

Kramer

Maldonado

Kimball

et al. 2020

Clinical Case Reports

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Section: Discussionsupporting

confidence: 79%

Flipping syncope: The case of an adolescent athlete with syncopal episodes ultimately diagnosed with catecholaminergic polymorphic ventricular tachycardia

Kramer

Maldonado

Kimball

et al. 2020

Clinical Case Reports

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“…The 2013 guidelines recommended (Class I) that a patient with CPVT has an ICD implanted if the patient has survived a cardiac arrest or if a patient has syncope/documented sustained VT despite optimal medical management and/or left cardiac sympathetic denervation (Al-Khatib et al 2018). A recent meta-analysis reviewed 53 studies containing 1429 CPVT patients, 35% of whom had an ICD implanted (Roston et al 2018a). During follow-up, 40% of patients received at least one appropriate shock, 21% of patients received at least one inappropriate shock, and electrical storm (three or more sustained episodes of ventricular tachycardia, ventricular fibrillation, or appropriate shocks from an ICD within 24 hours) occurred in 20% of patients.…”

Section: Implantable Cardiac Defibrillatormentioning

confidence: 99%

“…The effectiveness of the shocks was also assessed. Some 99% of shocks for ventricular tachycardia failed despite being appropriate whereas 94% of shocks for ventricular fibrillation were successful (Roston et al 2018a). Thus, the efficacy of ICD shocks in CPVT appears dependent on the arrhythmia mechanism -effective for ventricular fibrillation but ineffective for VT. (Miyake et al 2013) A recent multicenter study of 136 CPVT patients who presented with cardiac arrest showed no survival benefit associated with ICD implant (van der Werf et al 2019).…”

Section: Implantable Cardiac Defibrillatormentioning

confidence: 99%

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Wleklinski

Kannankeril

Knollmann

2020

The Journal of Physiology

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a stress-induced cardiac channelopathy that has a high mortality in untreated patients. Our understanding has grown tremendously since CPVT was first described as a clinical syndrome in 1995. It is now established that the deadly arrhythmias are caused by unregulated 'pathological' calcium release from the sarcoplasmic reticulum (SR), the major calcium storage organelle in striated muscle. Important questions remain regarding the molecular mechanisms that are responsible Matthew Wleklinski is an MD/PhD student interested in studying how mutations in calcium handling proteins lead to cardiac arrhythmias and sudden death. His current work focuses on the protein calsequestrin and its role in catecholaminergic polymorphic ventricular tachycardia. Dr

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“…ICDs have been shown to rescue patients with CPVT from cardiac arrest. About 47% of patients received an ICD for primary prevention, and during follow-up 40% had an appropriate shock as published by a study in 2018 [20]. The survival of undiagnosed CPVT patients with ICDs who presented with cardiac arrest was not improved [21].…”

Section: Discussionmentioning

confidence: 99%

Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia

Veith

El‐Battrawy

Roterberg

et al. 2020

JCM

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Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder causing life-threatening arrhythmias. Long-term outcome studies of the channelopathy are limited. Objective: The aim of the present study was to summarize our knowledge on CPVT patients, including the clinical profile treatment approach and long-term outcome. Methods: In this single center study, we retrospectively and prospectively collected data from nine CPVT patients and analyzed them. Results: We reviewed nine patients with CPVT in seven families (22% male), with a median follow-up time of 8.6 years. Mean age at diagnosis was 26.4 ± 12 years. Symptoms at admission were syncope (four patients) and aborted cardiac arrest (four patients). Family history of sudden cardiac death was screened in five patients. In genetic analyses, we found five patients with ryanodine type 2 receptor (RYR2) mutations. Seven patients were treated with beta-blockers, and if symptoms persisted flecainide was added (four patients). Despite beta-blocker treatment, three patients suffered from seven adverse cardiac events. An implantable cardioverter defibrillator was implanted in seven patients (one primary, six secondary prevention). Over the follow-up period, three patients suffered from ventricular tachycardia (ten times) and five patients from ventricular fibrillation (nine times). No one died during follow-up. Conclusion: Our CPVT cohort showed a high risk of cardiac events. Family screening, optimal medical therapy and individualized treatment are necessary in affected patients in referral centers.

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Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review

Cited by 90 publications

References 25 publications

Flipping syncope: The case of an adolescent athlete with syncopal episodes ultimately diagnosed with catecholaminergic polymorphic ventricular tachycardia

Flipping syncope: The case of an adolescent athlete with syncopal episodes ultimately diagnosed with catecholaminergic polymorphic ventricular tachycardia

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Long-Term Follow-Up of Patients with Catecholaminergic Polymorphic Ventricular Arrhythmia

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