Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification.

BACKGROUND AND AIMS: Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for potentially life-threatening arrhythmic events (AEs) even while treated with ß-blockers. The aim was to develop a model for individualized prediction of AEs in patients with RYR2-mediated...

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Detalles Bibliográficos
Autores: Lieve KV, van der Werf C, Kallas D, Denjoy I, Bos JM, Aiba T, Behr ER, van den Berg MP, Bergeman AT, Blom NA, Borggrefe M, Brugada R, Carrillo Mora LM, Chorin E, Crotti L, Davis A, Drago F, Dusi V, Extramiana F, Franciosi S, Giudicessi JR, González Llopis FÁ, Haugaa KH, van den Heuvel F, Horie M, Ingles J, Kammeraad J, Kannankeril PJ, Khan HR, Krahn AD, MacIntyre C, Maltret A, Marjamaa A, Ohno S, Peltenburg PJ, Perez GJ, Probst V, Roberts JD, Robyns T, Rootwelt-Norberg C, Roses I Noguer F, Roston TM, Rydberg A, Sacher F, Sarquella-Brugada G, Schwartz PJ, Semsarian C, Shimizu W, Starling L, Sumitomo N, Skinner JR, Tavacova T, Tfelt-Hansen J, Till JA, Yap SC, Wada Y, Wangüemert F, Zorio E, Ackerman MJ, Leenhardt A, Sanatani S, Tanck MW, Wilde AA
Tipo de recurso: artículo
Fecha de publicación:2025
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p29651
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29651
Access Level:acceso abierto
Palabra clave:Catecholaminergic polymorphic ventricular tachycardia
Risk stratification
Sudden cardiac death
Ventricular arrhythmias
ß-Blockers
Descripción
Sumario:BACKGROUND AND AIMS: Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for potentially life-threatening arrhythmic events (AEs) even while treated with ß-blockers. The aim was to develop a model for individualized prediction of AEs in patients with RYR2-mediated CPVT on ß-blocker monotherapy. METHODS: 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. RESULTS: 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 ß-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 ß-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. CONCLUSIONS: These externally validated risk prediction models using clinical parameters accurately distinguished CPVT patients on ß-blocker monotherapy at low and high risk for future AEs while treated with ß-blockers. These models provide guidance for implementation of clinical management therapies to prevent AEs in patients with CPVT.

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