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

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)1620-1627
Number of pages8
JournalPediatric Cardiology
Volume34
Issue number7
DOIs
StatePublished - Oct 2013

Fingerprint

Defibrillators
Hypertrophic Cardiomyopathy
Multicenter Studies
Shock
Sudden Cardiac Death
Implantable Defibrillators
Primary Prevention
Equipment and Supplies
Inborn Genetic Diseases
Incidence
Sudden Death
Secondary Prevention
Cohort Studies
Cardiovascular Diseases
Retrospective Studies

Keywords

  • Hypertrophic cardiomyopathy
  • Implantable cardioverter-defibrillator
  • Pediatric
  • Young adult

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Kamp, A. N., Von Bergen, N. H., Henrikson, C. A., Makhoul, M., Saarel, E. V., Lapage, M. J., ... Bradley, D. J. (2013). Implanted defibrillators in young hypertrophic cardiomyopathy patients: A multicenter study. Pediatric Cardiology, 34(7), 1620-1627. https://doi.org/10.1007/s00246-013-0676-6

Implanted defibrillators in young hypertrophic cardiomyopathy patients : A multicenter study. / Kamp, Anna N.; Von Bergen, Nicholas H.; Henrikson, Charles A.; Makhoul, Majd; Saarel, Elizabeth V.; Lapage, Martin J.; Russell, Mark W.; Strieper, Margaret; Yu, Sunkyung; Dick, Macdonald; Day, Sharlene M.; Bradley, David J.

In: Pediatric Cardiology, Vol. 34, No. 7, 10.2013, p. 1620-1627.

Research output: Contribution to journalArticle

Kamp, AN, Von Bergen, NH, Henrikson, CA, Makhoul, M, Saarel, EV, Lapage, MJ, Russell, MW, Strieper, M, Yu, S, Dick, M, Day, SM & Bradley, DJ 2013, 'Implanted defibrillators in young hypertrophic cardiomyopathy patients: A multicenter study', Pediatric Cardiology, vol. 34, no. 7, pp. 1620-1627. https://doi.org/10.1007/s00246-013-0676-6
Kamp AN, Von Bergen NH, Henrikson CA, Makhoul M, Saarel EV, Lapage MJ et al. Implanted defibrillators in young hypertrophic cardiomyopathy patients: A multicenter study. Pediatric Cardiology. 2013 Oct;34(7):1620-1627. https://doi.org/10.1007/s00246-013-0676-6
Kamp, Anna N. ; Von Bergen, Nicholas H. ; Henrikson, Charles A. ; Makhoul, Majd ; Saarel, Elizabeth V. ; Lapage, Martin J. ; Russell, Mark W. ; Strieper, Margaret ; Yu, Sunkyung ; Dick, Macdonald ; Day, Sharlene M. ; Bradley, David J. / Implanted defibrillators in young hypertrophic cardiomyopathy patients : A multicenter study. In: Pediatric Cardiology. 2013 ; Vol. 34, No. 7. pp. 1620-1627.
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