Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification
Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for potentially life-threatening arrhythmic events (AEs) even while treated with β-blockers. The aim was to develop a model for individualized prediction of AEs in patients with RYR2-mediated CPVT on β-blocker mon...
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
19 December 2025
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| In: |
European heart journal
Year: 2025, Pages: 1-12 |
| ISSN: | 1522-9645 |
| DOI: | 10.1093/eurheartj/ehaf965 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1093/eurheartj/ehaf965
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| Verfasserangaben: | Krystien V. Lieve, Christian van der Werf, Dania Kallas, Isabelle Denjoy, J. Martijn Bos, Takeshi Aiba, Elijah R. Behr, Maarten P. van den Berg, Auke T.Bergeman, Nico A. Blom, Martin Borggrefe, Ramon Brugada, Lidia María Carrillo Mora, Ehud Chorin, Lia Crotti, Andrew Davis, Fabrizio Drago, Veronica Dusi, Fabrice Extramiana, Sonia Franciosi, John R. Giudicessi, Francisco Ángel González Llopis, Kristina H. Haugaa, Freek van den Heuvel, Minoru Horie, Jodie Ingles, Janneke Kammeraad, Prince J. Kannankeril, Habib R. Khan, Andrew D. Krahn, Ciorsti MacIntyre, Alice Maltret, Annukka Marjamaa, Seiko Ohno, Puck J. Peltenburg, Guillermo J Perez, Vincent Probst, Jason D. Roberts, Tomas Robyns, Christine Rootwelt-Norberg, Ferran Roses I. Noguer, Thomas M. Roston, Annika Rydberg, Frederic Sacher, Georgia Sarquella-Brugada, Peter J. Schwartz, Christopher Semsarian, Wataru Shimizu, Luke Starling, Naokata Sumitomo, Jonathan R. Skinner, Terezia Tavacova, Jacob Tfelt-Hansen, Janice A. Till, Sing-Chien Yap, Yuko Wada, Fernando Wangüemert, Esther Zorio, Michael J. Ackerman, Antoine Leenhardt, Shubhayan Sanatani, Michael W. Tanck, and Arthur A. Wilde |
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| 245 | 1 | 0 | |a Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2 |b a validated risk stratification |c Krystien V. Lieve, Christian van der Werf, Dania Kallas, Isabelle Denjoy, J. Martijn Bos, Takeshi Aiba, Elijah R. Behr, Maarten P. van den Berg, Auke T.Bergeman, Nico A. Blom, Martin Borggrefe, Ramon Brugada, Lidia María Carrillo Mora, Ehud Chorin, Lia Crotti, Andrew Davis, Fabrizio Drago, Veronica Dusi, Fabrice Extramiana, Sonia Franciosi, John R. Giudicessi, Francisco Ángel González Llopis, Kristina H. Haugaa, Freek van den Heuvel, Minoru Horie, Jodie Ingles, Janneke Kammeraad, Prince J. Kannankeril, Habib R. Khan, Andrew D. Krahn, Ciorsti MacIntyre, Alice Maltret, Annukka Marjamaa, Seiko Ohno, Puck J. Peltenburg, Guillermo J Perez, Vincent Probst, Jason D. Roberts, Tomas Robyns, Christine Rootwelt-Norberg, Ferran Roses I. Noguer, Thomas M. Roston, Annika Rydberg, Frederic Sacher, Georgia Sarquella-Brugada, Peter J. Schwartz, Christopher Semsarian, Wataru Shimizu, Luke Starling, Naokata Sumitomo, Jonathan R. Skinner, Terezia Tavacova, Jacob Tfelt-Hansen, Janice A. Till, Sing-Chien Yap, Yuko Wada, Fernando Wangüemert, Esther Zorio, Michael J. Ackerman, Antoine Leenhardt, Shubhayan Sanatani, Michael W. Tanck, and Arthur A. Wilde |
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| 520 | |a Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for potentially life-threatening arrhythmic events (AEs) even while treated with β-blockers. The aim was to develop a model for individualized prediction of AEs in patients with RYR2-mediated CPVT on β-blocker monotherapy.The derivation and independent validation cohorts included 743 and 129 patients, respectively. AEs were defined as arrhythmic syncope, appropriate implantable cardioverter-defibrillator shock, sudden cardiac arrest (SCA), and sudden cardiac death. Near-fatal or fatal AEs (nf/fAEs) included all AEs except for arrhythmic syncope. Prediction models using Cox regression were developed and internally and externally validated.A total of 102 (13.7%) patients in the derivation cohort and 24 (18.6%) patients in the validation cohort experienced ≥1 AE over a median follow-up of 5.1 [interquartile range (IQR), 7.7] and 2.4 (IQR, 4.4) years, respectively. Predictors of AE were arrhythmic syncope or SCA prior to diagnosis and age at β-blocker initiation. In the derivation and validation cohorts, the optimism-corrected C-indices of the models for AE were 0.67 [95% confidence interval (CI) 0.62-0.72] and 0.59 (95% CI 0.48-0.71), respectively. For nf/fAEs, ventricular arrhythmia severity before β-blocker initiation was a fourth independent predictor, and C-indices of the models in the derivation and validation cohorts were 0.74 (95% CI 0.68-0.80) and 0.60 (95% CI 0.47-0.72), respectively. In the derivation cohort, calibration slopes were 1.00 (95% CI 0.59-1.41) for AE and 1.00 (95% CI 0.69-1.32) for nf/fAE.These externally validated risk prediction models using clinical parameters accurately distinguished CPVT patients on β-blocker monotherapy at low and high risk for future AEs while treated with β-blockers. These models provide guidance for implementation of clinical management therapies to prevent AEs in patients with CPVT. | ||
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