International multicenter cohort study on beta-blocker-free treatment strategies for catecholaminergic polymorphic ventricular tachycardia patients: ventricular tacchycardia
Background - Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, potentially life-threatening genetic heart disease. Nonselective beta-blockers (BBs) are highly effective in reducing CPVT-triggered arrhythmic events. However, some patients suffer from unacceptable BB side effects...
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| Main Authors: | , , , , , , , , , , , , , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
February 2025
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| In: |
JACC Clinical electrophysiology
Year: 2025, Volume: 11, Issue: 2, Pages: 270-278 |
| ISSN: | 2405-5018 |
| DOI: | 10.1016/j.jacep.2024.10.005 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.jacep.2024.10.005 Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S2405500X24008697 
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| Author Notes: | Raquel Neves, MD, Sahej Bains, BS, J. Martijn Bos, MD, P HD, Christian van der Werf, MD, PH D, Auke T. Bergeman, MD, Puck Peltenburg, MD, Nico A. Blom, MD, P HD, Shubhayan Sanatani, MD, Heikki Swan, MD, Vincent Probst, MD, Prince J. Kannankeril, MD, MSCI, Jonathan R. Skinner, MD, Ramon Brugada, MD, Tomas Robyns, MD, Martin Borggrefe, MD, Wataru Shimizu, MD, P HD, Janneke A.E. Kammeraad, MD, PH D, Andrew D. Krahn, MD, Arthur A.M. Wilde, MD, P HD, Michael J. Ackerman, MD, PHD |
| Summary: | Background - Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare, potentially life-threatening genetic heart disease. Nonselective beta-blockers (BBs) are highly effective in reducing CPVT-triggered arrhythmic events. However, some patients suffer from unacceptable BB side effects and might require strategies without a BB. - Objectives - This study sought to review the spectrum of and outcomes associated with BB-free treatment configurations in patients with CPVT enrolled in the International CPVT Registry. - Methods - From the Registry, patients with RYR2 variant-positive CPVT treated with a BB-free strategy for ≥6 months were included. Two treatment groups were defined: patients classified as very low risk and treated with intentional nontherapy (INT) and patients who needed to be treated but did not tolerate BBs and were treated with 3 different strategies. - Results - Overall, 100 of 1,017 patients (10%) were on a BB-free treatment strategy. There were 73 patients (33 females [42%]) in the INT group. In patients 66 (90%), INT was pursued after low-risk assessment in asymptomatic patients and absent or negligible stress test phenotype. Twenty-seven patients (22 females, 81%) were treated using 3 different BB-free treatment strategies (flecainide monotherapy, n = 21; left cardiac sympathetic denervation monotherapy, n = 2; flecainide + left cardiac sympathetic denervation, n = 4). In total, 25 patients (93%) were previously treated with BBs. During a median follow-up of 6 years (IQR: 3-9 years), 2 patients (2%) had a CPVT-associated event. - Conclusions - Although nonselective BBs remain the cornerstone treatment for CPVT, 10% of patients with CPVT required a BB-free treatment strategy. After careful risk assessment, safe and effective BB-free treatment strategies can be configured. |
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| Item Description: | Online verfügbar: 18. Dezember 2024, Artikelversion: 24. Februar 2025 Gesehen am 17.07.2025 |
| Physical Description: | Online Resource |
| ISSN: | 2405-5018 |
| DOI: | 10.1016/j.jacep.2024.10.005 |