Browsing by Subject "implantable cardioverter-defibrillator"

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    Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome.
    (JACC. Clinical electrophysiology, 2020-11) Moore, Jeremy P; Gallotti, Roberto G; Shannon, Kevin M; Bos, J Martijn; Sadeghi, Elham; Strasburger, Janette F; Wakai, Ronald T; Horigome, Hitoshi; Clur, Sally-Ann; Hill, Allison C; Shah, Maully J; Behere, Shashank; Sarquella-Brugada, Georgia; Czosek, Richard; Etheridge, Susan P; Fischbach, Peter; Kannankeril, Prince J; Motonaga, Kara; Landstrom, Andrew P; Williams, Matthew; Patel, Akash; Dagradi, Federica; Tan, Reina B; Stephenson, Elizabeth; Krishna, Mani Ram; Miyake, Christina Y; Lee, Michelle E; Sanatani, Shubhayan; Balaji, Seshadri; Young, Ming-Lon; Siddiqui, Saad; Schwartz, Peter J; Shivkumar, Kalyanam; Ackerman, Michael J

    Objectives

    This study sought to determine the relationship between long QT syndrome (LQTS) subtype (LTQ1, LTQ2, LTQ3) and postnatal cardiac events (CEs).

    Background

    LQTS presenting with 2:1 atrioventricular block or torsades de pointes in the fetus and/or neonate has been associated with risk for major CEs, but overall outcomes and predictors remain unknown.

    Methods

    A retrospective study involving 25 international centers evaluated the course of fetuses/newborns diagnosed with congenital LQTS and either 2:1 atrioventricular block or torsades de pointes. The primary outcomes were age at first CE after dismissal from the newborn hospitalization and death and/or cardiac transplantation during follow-up. CE was defined as aborted cardiac arrest, appropriate shock from implantable cardioverter-defibrillator, or sudden cardiac death.

    Results

    A total of 84 fetuses and/or neonates were identified with LQTS (12 as LQT1, 35 as LQT2, 37 as LQT3). Median gestational age at delivery was 37 weeks (interquartile range: 35 to 39 weeks) and age at hospital discharge was 3 weeks (interquartile range: 2 to 5 weeks). Fetal demise occurred in 2 and pre-discharge death in 1. Over a median of 5.2 years, there were 1 LQT1, 3 LQT2, and 23 LQT3 CEs (13 aborted cardiac arrests, 5 sudden cardiac deaths, and 9 appropriate shocks). One patient with LQT1 and 11 patients with LQT3 died or received cardiac transplant during follow-up. The only multivariate predictor of post-discharge CEs was LQT3 status (LQT3 vs. LQT2: hazard ratio: 8.4; 95% confidence interval: 2.6 to 38.9; p < 0.001), and LQT3, relative to LQT2, genotype predicted death and/or cardiac transplant (p < 0.001).

    Conclusions

    In this large multicenter study, fetuses and/or neonates with LQT3 but not those with LQT1 or LQT2 presenting with severe arrhythmias were at high risk of not only frequent, but lethal CEs.
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    The Use of Implantable Cardioverter-defibrillators in the Prevention of Sudden Cardiac Death: A Focus on Congenital Heart Disease and Inherited Arrhythmia Syndromes.
    (The Journal of innovations in cardiac rhythm management, 2018-01-15) Goldstein, Sarah A; Ward, Cary C; Al-Khatib, Sana M
    Some congenital heart diseases (CHDs) and inherited arrhythmia syndromes are associated with an increased risk of sudden cardiac death (SCD). Appropriate selection criteria for implantable cardioverter-defibrillator (ICD) implantation in these patients are poorly defined due to a paucity of data available from randomized clinical trials, leading to current guidelines relying more on non-randomized studies and expert opinions to make their recommendations. This review describes available evidence-based risk stratification methods for identifying patients at risk for SCD, as well as current guideline-driven management strategies for the use of ICDs in patients with CHD and inherited arrhythmia syndromes.