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
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...
April 6, 2024: Heart Rhythm: the Official Journal of the Heart Rhythm Society