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

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

Research output: Contribution to journalArticlepeer-review

25 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1791-1799
Number of pages9
JournalHeart rhythm
Issue number12
StatePublished - Dec 2018


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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)


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