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

Research output: Contribution to journal › Journal article › Research › peer-review

3 Citations (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 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.

Original language	English
Journal	Heart Rhythm
Volume	21
Issue number	10
Pages (from-to)	1767-1776
ISSN	1547-5271
DOIs	https://doi.org/10.1016/j.hrthm.2024.04.006
Publication status	Published - 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; 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, Vol. 21, No. 10, 2024, p. 1767-1776.

Research output: Contribution to journal › Journal article › Research › 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, vol. 21, no. 10, pp. 1767-1776. https://doi.org/10.1016/j.hrthm.2024.04.006

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. / An International Multicenter Cohort Study on Implantable Cardioverter Defibrillators for the Treatment of Symptomatic Children with Catecholaminergic Polymorphic Ventricular Tachycardia. In: Heart Rhythm. 2024 ; Vol. 21, No. 10. pp. 1767-1776.

@article{b31dcdf00fd746779d7be2d7344d5069,

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 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.",

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",

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

language = "English",

volume = "21",

pages = "1767--1776",

journal = "Heart Rhythm",

issn = "1547-5271",

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number = "10",

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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

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

VL - 21

SP - 1767

EP - 1776

JO - Heart Rhythm

JF - Heart Rhythm

SN - 1547-5271

IS - 10

ER -