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...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| 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 |
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Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification.Lieve KVvan der Werf CKallas DDenjoy IBos JMAiba TBehr ERvan den Berg MPBergeman ATBlom NABorggrefe MBrugada RCarrillo Mora LMChorin ECrotti LDavis ADrago FDusi VExtramiana FFranciosi SGiudicessi JRGonzález Llopis FÁHaugaa KHvan den Heuvel FHorie MIngles JKammeraad JKannankeril PJKhan HRKrahn ADMacIntyre CMaltret AMarjamaa AOhno SPeltenburg PJPerez GJProbst VRoberts JDRobyns TRootwelt-Norberg CRoses I Noguer FRoston TMRydberg ASacher FSarquella-Brugada GSchwartz PJSemsarian CShimizu WStarling LSumitomo NSkinner JRTavacova TTfelt-Hansen JTill JAYap SCWada YWangüemert FZorio EAckerman MJLeenhardt ASanatani STanck MWWilde AACatecholaminergic polymorphic ventricular tachycardiaRisk stratificationSudden cardiac deathVentricular arrhythmiasß-BlockersBACKGROUND 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.OXFORD UNIV PRESS2025info:eu-repo/semantics/articleaheadOfPrintVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29651EUROPEAN HEART JOURNALISSN: 0195668XISSNe: 15229645reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p296512026-05-27T12:37:41Z |
| dc.title.none.fl_str_mv |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| title |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| spellingShingle |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. Lieve KV Catecholaminergic polymorphic ventricular tachycardia Risk stratification Sudden cardiac death Ventricular arrhythmias ß-Blockers |
| title_short |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| title_full |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| title_fullStr |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| title_full_unstemmed |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| title_sort |
Catecholaminergic polymorphic ventricular tachycardia mediated by ryanodine receptor 2: a validated risk stratification. |
| dc.creator.none.fl_str_mv |
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 |
| author |
Lieve KV |
| author_facet |
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 |
| author_role |
author |
| author2 |
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 |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Catecholaminergic polymorphic ventricular tachycardia Risk stratification Sudden cardiac death Ventricular arrhythmias ß-Blockers |
| topic |
Catecholaminergic polymorphic ventricular tachycardia Risk stratification Sudden cardiac death Ventricular arrhythmias ß-Blockers |
| description |
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. |
| publishDate |
2025 |
| dc.date.none.fl_str_mv |
2025 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article aheadOfPrintVersion |
| format |
article |
| dc.identifier.none.fl_str_mv |
https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29651 |
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https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=29651 |
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Inglés |
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Inglés |
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info:eu-repo/semantics/openAccess |
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openAccess |
| dc.publisher.none.fl_str_mv |
OXFORD UNIV PRESS |
| publisher.none.fl_str_mv |
OXFORD UNIV PRESS |
| dc.source.none.fl_str_mv |
EUROPEAN HEART JOURNAL ISSN: 0195668X ISSNe: 15229645 reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu instname:Fundació Sant Joan de Déu |
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Fundació Sant Joan de Déu |
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r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
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r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu |
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15.811543 |