Elsevier

Heart Rhythm

Volume 13, Issue 2, February 2016, Pages 443-454
Heart Rhythm

Implantable cardioverter-defibrillator harm in young patients with inherited arrhythmia syndromes: A systematic review and meta-analysis of inappropriate shocks and complications

https://doi.org/10.1016/j.hrthm.2015.09.010Get rights and content

Background

Implantable cardioverter-defibrillators (ICDs) are implanted with the intention to prolong life in selected patients with inherited arrhythmia syndromes, but ICD implantation is also associated with inappropriate shocks and complications.

Objective

We aimed to quantify the rate of inappropriate shocks and other ICD-related complications to be able to weigh benefit and harm in these patients.

Methods

We performed a systematic review and meta-analysis of inappropriate shock and/or other ICD-related complication rates, including ICD-related mortality, in patients with inherited arrhythmia syndromes, that is, arrhythmogenic right ventricular cardiomyopathy/dysplasia, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, dilated cardiomyopathy due to a mutation in the lamin A/C gene, long QT syndrome, and short QT syndrome. We searched MEDLINE and EMBASE from inception to May 30, 2014.

Results

Of 2471 unique citations, 63 studies comprising 4916 patients with inherited arrhythmia syndromes (mean age of 39 ± 15 years) were included. Inappropriate shocks occurred in 20% of patients (crude annual rate of 4.7% per year), with a significantly higher rate in studies published before 2008 (6.1% per year vs 4.1% per year). Moreover, 22% experienced ICD-related complications (4.4% per year) and there was a 0.5% ICD-related mortality (0.08% per year).

Conclusion

ICD implantation carries a significant risk of inappropriate shocks and inhospital and postdischarge complications in relatively young patients with inherited arrhythmia syndromes. These data can be used to better inform patients and physicians about the expected risk of adverse ICD events and thereby facilitate shared decision making.

Introduction

The implantable cardioverter-defibrillator (ICD) is highly effective in reducing the rate of death due to ventricular tachyarrhythmia in high-risk patients with ischemic and nonischemic cardiomyopathy.1 Patients with inherited arrhythmia syndromes may also be at increased risk of sudden cardiac death (SCD) due to ventricular arrhythmias and can therefore benefit from ICDs as well.2 Increasing awareness and diagnosis of patients with a genetic predisposition for SCD has caused a huge growth of ICD implantation in this relatively young patient population.3

In contrast to the patients with ischemic and nonischemic cardiomyopathy with an ICD, appropriate selection criteria for prophylactic ICD implantation in patients with inherited arrhythmia syndromes rely on nonrandomized studies and consensus statements.4 While the presumed benefit of ICDs is reflected in the consensus statements, the associated harm has received less attention so far. However, quantification of the risk of ICD therapy is needed to obtain truly informed consent. Inappropriate shocks and device-related complications may actually be more prevalent in these predominantly young patients because of an active lifestyle. Moreover, patients with inherited arrhythmia syndromes face many decades of ICD therapy and thus they will be exposed to the risks of ICD therapy for a longer period, increasing the cumulative prevalence of ICD harm. This does not imply that ICD implantation should simply be deferred in patients with a high risk of complications, but certainly warrants weighing the benefit and harm in patients where evidence of benefit is more limited.

We therefore performed a systematic review of observational studies quantifying the occurrence of ICD harm in patients with inherited arrhythmia syndromes at increased risk of SCD in an effort to examine the rate of inappropriate shocks and ICD-related complications including ICD-related mortality, as this crucial information is currently lacking. This information on the safety of ICD therapy in these patient populations is needed to create a reasonable expectation of procedural and long-term risk-benefit ratios.

Section snippets

Search strategy and selection criteria

We performed this meta-analysis in accordance with the Meta-analysis Of Observational Studies in Epidemiology guidelines for observational cohort studies and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines for reporting. A clinical librarian (J.L.) performed an extensive electronic search in MEDLINE (Ovid) and EMBASE (Ovid) from inception to May 30, 2014. In addition, the PubMed publisher subset was searched to find publications not yet included in MEDLINE. The

Search results

Of 2471 articles the literature search yielded, 63 cohort studies were included after review, exclusion, and cross-referencing (Figure 1).2, 3, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64 In total, the analysis included 4916 patients with inherited arrhythmia syndromes: 710 (14%) with ARVC, 1037 (21%) with

Main results

In this meta-analysis of data from 63 study populations comprising 4916 young patients (mean age 39 ± 15 years) with inherited arrhythmia syndromes with an ICD, inappropriate shocks occurred in 20% of patients (crude annual rate 4.7% per year). Various other types of ICD-related complications occurred in 22% of patients (4.4% per year) and ICD-related mortality was 0.5% (0.08% per year). The risk of appropriate shocks varies among the different phenotypes, which might be ascribed to the

Conclusion

Since the decision to implant an ICD in patients with inherited arrhythmia syndromes represents a profound lifelong decision and commitment, informed consent is mandatory in light of its potential complications. This review shows that there are significant negative outcomes in terms of both morbidity and, at times, mortality associated with these devices. Inappropriate shocks occurred in 20% of patients (crude annual rate 4.7% per year). Moreover, various other types of ICD-related

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    Dr Wilde received support from the Netherlands CardioVascular Research Initiative of the Netherlands Heart Foundation: the Dutch Heart Foundation, the Dutch Federation of University Medical Centres, the Netherlands Organisation for Health Research and Development, and the Royal Netherlands Academy of Sciences. Dr de Groot was supported by a personal grant from the Netherlands Organization for Scientific Research (NWO/ZonMW grant no. 016.146.310).

    Dr de Groot has received research grant from St Jude Medical, Medtronic, AtriCure and is a consultant to AtriCure and Daiichi Sankyo.

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