An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia

Avani Lamba; Thomas M Roston; Puck J Peltenburg; Dania Kallas; Sonia Franciosi; Krystien V V Lieve; Prince J Kannankeril; Minoru Horie; Seiko Ohno; Ramon Brugada; Takeshi Aiba; Peter Fischbach; Linda Knight; Jan Till; Sit-Yee Kwok; Vincent Probst; David Backhoff; Martin J LaPage; Anjan S Batra; Fabrizio Drago; Kristina Haugaa; Andrew D Krahn; Tomas Robyns; Heikki Swan; Terezia Tavacova; Joseph Atallah; Martin Borggrefe; Boris Rudic; Georgia Sarquella-Brugada; Ehud Chorin; Allison Hill; Janneke Kammeraad; Anna Kamp; Ian Law; James Perry; Jason D Roberts; Svjetlana Tisma-Dupanovic; Christopher Semsarian; Jonathan R Skinner; Jacob Tfelt-Hansen; Isabelle Denjoy; Antoine Leenhardt; Peter J Schwartz; Michael J Ackerman; Arthur A M Wilde; Christian van der Werf; Shubhayan Sanatani

doi:10.1016/j.hrthm.2024.04.006

An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia

Avani Lamba, Thomas M Roston, Puck J Peltenburg, Dania Kallas, Sonia Franciosi, Krystien V V Lieve, Prince J Kannankeril, Minoru Horie, Seiko Ohno, Ramon Brugada, Takeshi Aiba, Peter Fischbach, Linda Knight, Jan Till, Sit-Yee Kwok, Vincent Probst, David Backhoff, Martin J LaPage, Anjan S Batra, Fabrizio DragoKristina Haugaa, Andrew D Krahn, Tomas Robyns, Heikki Swan, Terezia Tavacova, Joseph Atallah, Martin Borggrefe, Boris Rudic, Georgia Sarquella-Brugada, Ehud Chorin, Allison Hill, Janneke Kammeraad, Anna Kamp, Ian Law, James Perry, Jason D Roberts, Svjetlana Tisma-Dupanovic, Christopher Semsarian, Jonathan R Skinner, Jacob Tfelt-Hansen, Isabelle Denjoy, Antoine Leenhardt, Peter J Schwartz, Michael J Ackerman, Arthur A M Wilde, Christian van der Werf, Shubhayan Sanatani^*

^*Corresponding author af dette arbejde

Afdeling for Hjertesygdomme

6 Citationer (Scopus)

Abstract

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 is limited data on the outcomes of ICD use in children.

OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.

METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.

RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.

CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.

Originalsprog	Engelsk
Tidsskrift	Heart Rhythm
Vol/bind	21
Udgave nummer	10
Sider (fra-til)	1767-1776
Antal sider	10
ISSN	1547-5271
DOI	https://doi.org/10.1016/j.hrthm.2024.04.006
Status	Udgivet - okt. 2024

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10.1016/j.hrthm.2024.04.006

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Lamba, A., Roston, T. M., Peltenburg, P. J., Kallas, D., Franciosi, S., Lieve, K. V. V., Kannankeril, P. J., Horie, M., Ohno, S., Brugada, R., Aiba, T., Fischbach, P., Knight, L., Till, J., Kwok, S.-Y., Probst, V., Backhoff, D., LaPage, M. J., Batra, A. S., ... Sanatani, S. (2024). An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia. Heart Rhythm, 21(10), 1767-1776. https://doi.org/10.1016/j.hrthm.2024.04.006

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 et al.
I: Heart Rhythm, Bind 21, Nr. 10, 10.2024, s. 1767-1776.

Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › peer review

Lamba, A, Roston, TM, Peltenburg, PJ, Kallas, D, Franciosi, S, Lieve, KVV, Kannankeril, PJ, Horie, M, Ohno, S, Brugada, R, Aiba, T, Fischbach, P, Knight, L, Till, J, Kwok, S-Y, Probst, V, Backhoff, D, LaPage, MJ, Batra, AS, Drago, F, Haugaa, K, Krahn, AD, Robyns, T, Swan, H, Tavacova, T, Atallah, J, Borggrefe, M, Rudic, B, Sarquella-Brugada, G, Chorin, E, Hill, A, Kammeraad, J, Kamp, A, Law, I, Perry, J, Roberts, JD, Tisma-Dupanovic, S, Semsarian, C, Skinner, JR, Tfelt-Hansen, J, Denjoy, I, Leenhardt, A, Schwartz, PJ, Ackerman, MJ, Wilde, AAM, van der Werf, C & Sanatani, S 2024, 'An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia', Heart Rhythm, bind 21, nr. 10, s. 1767-1776. https://doi.org/10.1016/j.hrthm.2024.04.006

@article{06d22d25d504449daea14ea114912e83,

title = "An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia",

abstract = "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 is limited data on the outcomes of ICD use in children.OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.",

keywords = "Adolescent, Child, Child, Preschool, Death, Sudden, Cardiac/prevention & control, Defibrillators, Implantable, Female, Follow-Up Studies, Humans, Male, Retrospective Studies, Ryanodine Receptor Calcium Release Channel/genetics, Tachycardia, Ventricular/therapy, Treatment Outcome",

author = "Avani Lamba and Roston, {Thomas M} and Peltenburg, {Puck J} and Dania Kallas and Sonia Franciosi and Lieve, {Krystien V V} and Kannankeril, {Prince J} and Minoru Horie and Seiko Ohno and Ramon Brugada and Takeshi Aiba and Peter Fischbach and Linda Knight and Jan Till and Sit-Yee Kwok and Vincent Probst and David Backhoff and LaPage, {Martin J} and Batra, {Anjan S} and Fabrizio Drago and Kristina Haugaa and Krahn, {Andrew D} and Tomas Robyns and Heikki Swan and Terezia Tavacova and Joseph Atallah and Martin Borggrefe and Boris Rudic and Georgia Sarquella-Brugada and Ehud Chorin and Allison Hill and Janneke Kammeraad and Anna Kamp and Ian Law and James Perry and Roberts, {Jason D} and Svjetlana Tisma-Dupanovic and Christopher Semsarian and Skinner, {Jonathan R} and Jacob Tfelt-Hansen and Isabelle Denjoy and Antoine Leenhardt and Schwartz, {Peter J} and Ackerman, {Michael J} and Wilde, {Arthur A M} and {van der Werf}, Christian and Shubhayan Sanatani",

note = "Copyright {\textcopyright} 2024. Published by Elsevier Inc.",

year = "2024",

month = oct,

doi = "10.1016/j.hrthm.2024.04.006",

language = "English",

volume = "21",

pages = "1767--1776",

journal = "Heart Rhythm",

issn = "1547-5271",

publisher = "Elsevier BV",

number = "10",

}

TY - JOUR

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

PY - 2024/10

Y1 - 2024/10

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 is limited data on the outcomes of ICD use in children.OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs 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 is limited data on the outcomes of ICD use in children.OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD.METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope.RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications.CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.

KW - Adolescent

KW - Child

KW - Child, Preschool

KW - Death, Sudden, Cardiac/prevention & control

KW - Defibrillators, Implantable

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Male

KW - Retrospective Studies

KW - Ryanodine Receptor Calcium Release Channel/genetics

KW - Tachycardia, Ventricular/therapy

KW - Treatment Outcome

UR - http://www.scopus.com/inward/record.url?scp=85196031851&partnerID=8YFLogxK

U2 - 10.1016/j.hrthm.2024.04.006

DO - 10.1016/j.hrthm.2024.04.006

M3 - Journal article

C2 - 38588993

SN - 1547-5271

VL - 21

SP - 1767

EP - 1776

JO - Heart Rhythm

JF - Heart Rhythm

IS - 10

ER -

An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia

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