Implanted defibrillators in young hypertrophic cardiomyopathy patients

A multicenter study

Anna N. Kamp, Nicholas H. Von Bergen, Charles 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

    9 Citations (Scopus)

    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., 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; 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, C, 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, Anna N. ; Von Bergen, Nicholas H. ; Henrikson, Charles ; 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.
    @article{5814700bdbf34bd08aa898d584a3a84f,
    title = "Implanted defibrillators in young hypertrophic cardiomyopathy patients: A multicenter study",
    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",
    author = "Kamp, {Anna N.} and {Von Bergen}, {Nicholas H.} and Charles Henrikson and Majd Makhoul and Saarel, {Elizabeth V.} and Lapage, {Martin J.} and Russell, {Mark W.} and Margaret Strieper and Sunkyung Yu and Macdonald Dick and Day, {Sharlene M.} and Bradley, {David J.}",
    year = "2013",
    month = "10",
    doi = "10.1007/s00246-013-0676-6",
    language = "English (US)",
    volume = "34",
    pages = "1620--1627",
    journal = "Pediatric Cardiology",
    issn = "0172-0643",
    publisher = "Springer New York",
    number = "7",

    }

    TY - JOUR

    T1 - Implanted defibrillators in young hypertrophic cardiomyopathy patients

    T2 - A multicenter study

    AU - Kamp, Anna N.

    AU - Von Bergen, Nicholas H.

    AU - Henrikson, Charles

    AU - Makhoul, Majd

    AU - Saarel, Elizabeth V.

    AU - Lapage, Martin J.

    AU - Russell, Mark W.

    AU - Strieper, Margaret

    AU - Yu, Sunkyung

    AU - Dick, Macdonald

    AU - Day, Sharlene M.

    AU - Bradley, David J.

    PY - 2013/10

    Y1 - 2013/10

    N2 - 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.

    AB - 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.

    KW - Hypertrophic cardiomyopathy

    KW - Implantable cardioverter-defibrillator

    KW - Pediatric

    KW - Young adult

    UR - http://www.scopus.com/inward/record.url?scp=84885387979&partnerID=8YFLogxK

    UR - http://www.scopus.com/inward/citedby.url?scp=84885387979&partnerID=8YFLogxK

    U2 - 10.1007/s00246-013-0676-6

    DO - 10.1007/s00246-013-0676-6

    M3 - Article

    VL - 34

    SP - 1620

    EP - 1627

    JO - Pediatric Cardiology

    JF - Pediatric Cardiology

    SN - 0172-0643

    IS - 7

    ER -