An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia
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An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia. / Lamba, Avani; Roston, Thomas M; Peltenburg, Puck J; Kallas, Dania; Franciosi, Sonia; Lieve, Krystien V V; Kannankeril, Prince J; Horie, Minoru; Ohno, Seiko; Brugada, Ramon; Aiba, Takeshi; Fischbach, Peter; Knight, Linda; Till, Jan; Kwok, Sit-Yee; Probst, Vincent; Backhoff, David; LaPage, Martin J; Batra, Anjan S; Drago, Fabrizio; Haugaa, Kristina; Krahn, Andrew D; Robyns, Tomas; Swan, Heikki; Tavacova, Terezia; Atallah, Joseph; Borggrefe, Martin; Rudic, Boris; Sarquella-Brugada, Georgia; Chorin, Ehud; Hill, Allison; Kammeraad, Janneke; Kamp, Anna; Law, Ian; Perry, James; Roberts, Jason D; Tisma-Dupanovic, Svjetlana; Semsarian, Christopher; Skinner, Jonathan R; Tfelt-Hansen, Jacob; Denjoy, Isabelle; Leenhardt, Antoine; Schwartz, Peter J; Ackerman, Michael J; Wilde, Arthur A M; van der Werf, Christian; Sanatani, Shubhayan.
In: Heart Rhythm, 2024.Research output: Contribution to journal › Journal article › Research › peer-review
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T1 - An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia
AU - Lamba, Avani
AU - Roston, Thomas M
AU - Peltenburg, Puck J
AU - Kallas, Dania
AU - Franciosi, Sonia
AU - Lieve, Krystien V V
AU - Kannankeril, Prince J
AU - Horie, Minoru
AU - Ohno, Seiko
AU - Brugada, Ramon
AU - Aiba, Takeshi
AU - Fischbach, Peter
AU - Knight, Linda
AU - Till, Jan
AU - Kwok, Sit-Yee
AU - Probst, Vincent
AU - Backhoff, David
AU - LaPage, Martin J
AU - Batra, Anjan S
AU - Drago, Fabrizio
AU - Haugaa, Kristina
AU - Krahn, Andrew D
AU - Robyns, Tomas
AU - Swan, Heikki
AU - Tavacova, Terezia
AU - Atallah, Joseph
AU - Borggrefe, Martin
AU - Rudic, Boris
AU - Sarquella-Brugada, Georgia
AU - Chorin, Ehud
AU - Hill, Allison
AU - Kammeraad, Janneke
AU - Kamp, Anna
AU - Law, Ian
AU - Perry, James
AU - Roberts, Jason D
AU - Tisma-Dupanovic, Svjetlana
AU - Semsarian, Christopher
AU - Skinner, Jonathan R
AU - Tfelt-Hansen, Jacob
AU - Denjoy, Isabelle
AU - Leenhardt, Antoine
AU - Schwartz, Peter J
AU - Ackerman, Michael J
AU - Wilde, Arthur A M
AU - van der Werf, Christian
AU - Sanatani, Shubhayan
N1 - Copyright © 2024. Published by Elsevier Inc.
PY - 2024
Y1 - 2024
N2 - 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 <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<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.
AB - 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 <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<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.
U2 - 10.1016/j.hrthm.2024.04.006
DO - 10.1016/j.hrthm.2024.04.006
M3 - Journal article
C2 - 38588993
JO - Heart Rhythm
JF - Heart Rhythm
SN - 1547-5271
ER -
ID: 389849479