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

Thomas M Roston¹, Karolina Jones², Nathaniel M Hawkins³

¹Department of Medicine, University of Alberta, Edmonton, Alberta, Canada; BC Children's Hospital, Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada.

|August 1, 2018

Insights

Implantable cardioverter-defibrillators (ICDs) are frequently used in catecholaminergic polymorphic ventricular tachycardia (CPVT) patients, but lead to significant shocks and complications. Optimizing antiarrhythmic therapy could reduce ICD necessity and associated harms.

Area of Science:

Cardiology
Electrophysiology
Genetics

Background:

Catecholaminergic polymorphic ventricular tachycardia (CPVT) poses a high risk for complications with implantable cardioverter-defibrillators (ICDs).
Systematic evaluation of ICD use and outcomes in CPVT patients is lacking.

Purpose of the Study:

To characterize the utilization and clinical outcomes of ICDs in patients diagnosed with CPVT.

Main Methods:

A systematic review was performed across Embase, MEDLINE, PubMed, and Google Scholar.
Studies involving CPVT patients with ICDs were identified and analyzed.

Main Results:

The review included 53 studies with 1429 CPVT patients; 503 (35.2%) received ICDs, often for primary prevention (47.3%).
ICD recipients experienced high rates of appropriate (40.1%) and inappropriate shocks (20.8%), electrical storm (19.6%), and complications (32.4%).
Adjuvant therapies like beta-blockers and flecainide were underutilized, with only 12.8% receiving optimal antiarrhythmic therapy.

Conclusions:

ICDs in CPVT patients are associated with substantial shocks and complications.
Increased adherence to guideline-directed management, including optimal antiarrhythmic therapies, may decrease ICD use and associated harm.

Read full analysis

Abstract

Background:

The implantable cardioverter-defibrillator (ICD) may be associated with a high risk of complications in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). However, ICDs in this population have not been systematically evaluated.

Objective:

The purpose of this study was to characterize the use and outcomes of ICDs in CPVT.

Methods:

We conducted a systematic review using Embase, MEDLINE, PubMed, and Google Scholar to identify studies that included patients with CPVT who had an ICD.

Results:

Fifty-three studies describing 1429 patients with CPVT were included. In total, 503 patients (35.2%) had an ICD (median age 15.0 years; interquartile range 11.0-21.0 years). Among ICD recipients with a reported medication status, 96.7% were prescribed β-blockers and 13.2% flecainide. Sympathetic denervation was performed in 23.2%. Nearly half of patients received an ICD for primary prevention (47.3%), and 12.8% were prescribed optimal antiarrhythmic therapy. During follow-up, 40.1% had ≥1 appropriate shock, 20.8% experienced ≥1 inappropriate shock, 19.6% had electrical storm, and 7 patients (1.4%) died. An ICD-associated electrical storm was implicated in 4 deaths. Additional complications such as lead failure, endocarditis, or surgical revisions were observed in 96 of 296 patients (32.4%). A subanalysis of the 10 studies encompassing 330 patients with the most detailed ICD-related data showed similar trends.

Conclusion:

In this population with CPVT, ICDs were common and associated with a high burden of shocks and complications. The reliance on primary prevention ICDs, and poor uptake of adjuvant antiarrhythmic therapies, suggests that improved adherence to guideline-directed management could reduce ICD use and harm.

Keywords:

Catecholaminergic polymorphic ventricular tachycardia Flecainide Implantable cardioverter-defibrillator Sudden cardiac death Sympathetic denervation

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