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

Heart Rhythm. 2016 Feb;13(2):443-54. doi: 10.1016/j.hrthm.2015.09.010. Epub 2015 Sep 15.


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.

Keywords: Complication; Genetics; Implantable cardioverter-defibrillator; Inappropriate shock; Inherited arrhythmia syndromes.

Publication types

  • Meta-Analysis
  • Research Support, Non-U.S. Gov't
  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Arrhythmias, Cardiac* / complications
  • Arrhythmias, Cardiac* / congenital
  • Arrhythmias, Cardiac* / therapy
  • Death, Sudden, Cardiac / etiology
  • Death, Sudden, Cardiac / prevention & control*
  • Defibrillators, Implantable / adverse effects*
  • Electric Countershock* / adverse effects
  • Electric Countershock* / instrumentation
  • Electric Countershock* / methods
  • Equipment Failure Analysis
  • Humans
  • Middle Aged
  • Risk Assessment