Pediatric Cardiology

, Volume 34, Issue 7, pp 1620–1627

Implanted Defibrillators in Young Hypertrophic Cardiomyopathy Patients: A Multicenter Study

  • Anna N. Kamp
  • Nicholas H. Von Bergen
  • Charles A. Henrikson
  • Majd Makhoul
  • Elizabeth V. Saarel
  • Martin J. LaPage
  • Mark W. Russell
  • Margaret Strieper
  • Sunkyung Yu
  • Macdonald Dick
  • Sharlene M. Day
  • David J. Bradley
Original Article

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease, with an annual risk of sudden cardiac death (SCD) estimated at 1 %. Limited data are available regarding both the risk of SCD in the young HCM population and the use of implantable cardioverter-defibrillators (ICDs). This retrospective study included all patients with HCM who underwent ICD implantation for primary or secondary prevention of SCD before the age of 30 years at five institutions between 1995 and 2009. There were 99 devices implanted in 73 patients. Appropriate shocks occurred for 11 % of all the patients. None of the previously identified conventional risk factors for SCD in HCM patients were associated with increased risk of appropriate shocks in the young study cohort. During a median follow-up period of 2.4 years, inappropriate shocks occurred for 22 % of the patients. Older age at implant was associated with a decreased risk of inappropriate shock. Those who underwent implantation in the earlier decade had a higher incidence of inappropriate shocks. Late complications including lead fracture or dislodgement, generator malfunction, and infection occurred for 32 % of the patients. Three patients died (4 %), one of whom had an arrhythmic sudden death. A greater proportion of primary prevention implantations was performed for patients from the latter decade. Over time, ICD use in young HCM patients has become increasingly primary prevention oriented. Shock rates mirror those reported in adult series, and there is a substantial incidence of device complications.

Keywords

Hypertrophic cardiomyopathy Implantable cardioverter-defibrillator Pediatric Young adult 

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Anna N. Kamp
    • 1
  • Nicholas H. Von Bergen
    • 2
  • Charles A. Henrikson
    • 3
    • 4
  • Majd Makhoul
    • 5
  • Elizabeth V. Saarel
    • 6
  • Martin J. LaPage
    • 7
  • Mark W. Russell
    • 7
  • Margaret Strieper
    • 5
  • Sunkyung Yu
    • 7
  • Macdonald Dick
    • 7
  • Sharlene M. Day
    • 7
    • 8
  • David J. Bradley
    • 7
  1. 1.MN150 Chandler Medical CenterUniversity of KentuckyLexingtonUSA
  2. 2.UI Children’s Hospitals Pediatric Specialty ClinicsUniversity of IowaIowa CityUSA
  3. 3.Johns HopkinsBaltimoreUSA
  4. 4.Division of Cardiovascular Medicine, MC UHN-62Oregon Health & Science UniversityPortlandUSA
  5. 5.Sibley Heart Center CardiologyEmory UniversityAtlantaUSA
  6. 6.University of UtahSalt Lake CityUSA
  7. 7.CS Mott Children’s Hospital Floor 11 Room 661University of MichiganAnn ArborUSA
  8. 8.Cardiovascular Center 1500 East Medical Center DriveAnn ArborUSA

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