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An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F24%3A10479281" target="_blank" >RIV/00064203:_____/24:10479281 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/24:10479281

  • Result on the web

    <a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=.gQs2Mrujc" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=.gQs2Mrujc</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1016/j.hrthm.2024.04.006" target="_blank" >10.1016/j.hrthm.2024.04.006</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia

  • Original language description

    BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter defibrillators (ICDs) are commonly advised. However, there are limited data on the outcomes of ICD use in children. OBJECTIVE: To compare the risk of arrhythmic events in pediatric CPVT patients with and without ICDs. METHODS: We compared the risk of SCD in RYR2 variant and phenotype positive symptomatic CPVT patients with and without ICDs, who were &lt;19 years of age and had no history of sudden cardiac arrest (SCA) at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite incidences of SCD, SCA, appropriate ICD shocks, with/without arrhythmic syncope. RESULTS: The study included 235 patients, 73 (31.1%) with ICDs and 162 (68.9%) without ICDs. Over a median follow-up of 8.0 years (IQR 4.3-13.4), SCD occurred in 7 (3.0%) patients, of which 4 (57.1%) were non-compliant with medications and none had an ICD. ICD patients had a higher risk of both secondary composite outcomes (without syncope: HR 5.85 (CI 3.40-10.09); p&lt;0.0001; with syncope: HR 2.55 (CI 1.50-4.34); p=0.0005). Thirty-one (42.5%) patients with an ICD experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications. CONCLUSIONS: SCD events occurred only in the no ICD group, in those not on optimal medical therapy. ICD patients had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common and risks versus benefits of ICDs need to be considered.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30201 - Cardiac and Cardiovascular systems

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2024

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Heart Rhythm

  • ISSN

    1547-5271

  • e-ISSN

    1556-3871

  • Volume of the periodical

    21

  • Issue of the periodical within the volume

    10

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    10

  • Pages from-to

    1767-1776

  • UT code for WoS article

    001343342600001

  • EID of the result in the Scopus database

    2-s2.0-85196031851