Moisés Rodríguez-Mañero1, Aurora Baluja2, Jaime Hernández3, Carmen Muñoz4, David Calvo5, Juan Fernández-Armenta6, Amaya García-Fernández7, Esther Zorio8, Álvaro Arce-León9, Juan Miguel Sánchez-Gómez10, Ignacio Mosquera-Pérez11, Miguel Á Arias12, Ernesto Díaz-Infante13, Víctor Expósito14, Víctor Jiménez-Ramos15, Elvis Teijeira16, María Victoria Cañadas-Godoy17, José María Guerra-Ramos18, Teresa Oloriz19, Nuria Basterra20, Pedro Sousa21, Juliana Elices-Teja22, Ignacio García-Bolao23, José Ramón González-Juanatey24, Ramón Brugada25, Juan Ramón Gimeno26, Josep Brugada27, Elena Arbelo27. 1. Servicio de Cardiología, Hospital Universitario Santiago de Compostela, Santiago de Compostela, A Coruña, Spain; Instituto de Investigación Sanitaria (IDIS), Universidad de Santiago de Compostela, Santiago de Compostela, A Coruña, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain. Electronic address: moirmanero@gmail.com. 2. Servicio de Anestesiología, Hospital Universitario Santiago de Compostela, Santiago de Compostela, A Coruña, Spain. 3. Sección de Arritmias, Servicio de Cardiología, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain; IDIBAPS, Institut d'Investigació August Pi i Sunyer (IDIBAPS), Barcelona, Spain. 4. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain; Servicio de Cardiología, Hospital Universitario de Murcia, Murcia, Spain. 5. Servicio de Cardiología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain. 6. Servicio de Cardiología, Hospital Universitario Puerta del Mar, Cádiz, Spain. 7. Servicio de Cardiología, Hospital Universitario General de Alicante, Alicante, Spain. 8. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain; Servicio de Cardiología, Hospital Universitario La Fe, Valencia, Spain. 9. Servicio de Cardiología, Hospital Universitario Juan Ramón Jiménez, Huelva, Spain. 10. Servicio de Cardiología, Hospital Universitario Clínico de Valencia, Valencia, Spain. 11. Servicio de Cardiología, Complejo Hospitalario Canalejo, A Coruña, Spain. 12. Servicio de Cardiología, Hospital Universitario Virgen de la Salud, Toledo, Spain. 13. Servicio de Cardiología, Hospital Universitario Virgen de la Macarena, Seville, Spain. 14. Servicio de Cardiología, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain. 15. Servicio de Cardiología, Hospital Universitario Santiago de Compostela, Santiago de Compostela, A Coruña, Spain. 16. Servicio de Cardiología, Hospital Universitario Vigo, Vigo, Pontevedra, Spain. 17. Servicio de Cardiología, Hospital Universitario Clínico de Madrid, Madrid, Spain. 18. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain; Servicio de Cardiología, Hospital Universitario Sant Pau de Barcelona, Barcelona, Spain. 19. Servicio de Cardiología, Hospital Universitario Clínico de Zaragoza, Zaragoza, Spain. 20. Servicio de Cardiología, Hospital Universitario de Navarra, Pamplona, Navarra, Spain. 21. Departamento de Cardiología, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. 22. Servicio de Cardiología, Hospital Universitario Lucus Augusti, Lugo, Spain. 23. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain; Servicio de Cardiología, Clínica Universidad de Navarra, Pamplona, Navarra, Spain. 24. Servicio de Cardiología, Hospital Universitario Santiago de Compostela, Santiago de Compostela, A Coruña, Spain; Instituto de Investigación Sanitaria (IDIS), Universidad de Santiago de Compostela, Santiago de Compostela, A Coruña, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain. 25. Centro de Genética Cardiovascular, Universidad of Girona, Girona, Spain. 26. Servicio de Cardiología, Hospital Universitario de Murcia, Murcia, Spain. 27. Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain; Sección de Arritmias, Servicio de Cardiología, Hospital Clínic, Universitat de Barcelona, Barcelona, Spain; IDIBAPS, Institut d'Investigació August Pi i Sunyer (IDIBAPS), Barcelona, Spain; The European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart - ERN GUARD-Heart.
Abstract
INTRODUCTION AND OBJECTIVES: Multiparametric scores have been designed for better risk stratification in Brugada syndrome (BrS). We aimed to validate 3 multiparametric approaches (the Delise score, Sieira score and the Shanghai BrS Score) in a cohort with Brugada syndrome and electrophysiological study (EPS). METHODS: We included patients diagnosed with BrS and previous EPS between 1998 and 2019 in 23 hospitals. C-statistic analysis and Cox proportional hazard regression models were used. RESULTS: A total of 831 patients were included (mean age, 42.8±13.1; 623 [75%] men; 386 [46.5%] had a type 1 electrocardiogram (ECG) pattern, 677 [81.5%] were asymptomatic, and 319 [38.4%] had an implantable cardioverter-defibrillator). During a follow-up of 10.2±4.7 years, 47 (5.7%) experienced a cardiovascular event. In the global cohort, a type 1 ECG and syncope were predictive of arrhythmic events. All risk scores were significantly associated with events. The discriminatory abilities of the 3 scores were modest (particularly when these scores were evaluated in asymptomatic patients). Evaluation of the Delise and Sieira scores with different numbers of extra stimuli (1 or 2 vs 3) did not substantially improve the event prediction c-index. CONCLUSIONS: In BrS, classic risk factors such as ECG pattern and previous syncope predict arrhythmic events. The predictive capabilities of the EPS are affected by the number of extra stimuli required to induce ventricular arrhythmias. Scores combining clinical risk factors with EPS help to identify the populations at highest risk, although their predictive abilities remain modest in the general BrS population and in asymptomatic patients.
INTRODUCTION AND OBJECTIVES: Multiparametric scores have been designed for better risk stratification in Brugada syndrome (BrS). We aimed to validate 3 multiparametric approaches (the Delise score, Sieira score and the Shanghai BrS Score) in a cohort with Brugada syndrome and electrophysiological study (EPS). METHODS: We included patients diagnosed with BrS and previous EPS between 1998 and 2019 in 23 hospitals. C-statistic analysis and Cox proportional hazard regression models were used. RESULTS: A total of 831 patients were included (mean age, 42.8±13.1; 623 [75%] men; 386 [46.5%] had a type 1 electrocardiogram (ECG) pattern, 677 [81.5%] were asymptomatic, and 319 [38.4%] had an implantable cardioverter-defibrillator). During a follow-up of 10.2±4.7 years, 47 (5.7%) experienced a cardiovascular event. In the global cohort, a type 1 ECG and syncope were predictive of arrhythmic events. All risk scores were significantly associated with events. The discriminatory abilities of the 3 scores were modest (particularly when these scores were evaluated in asymptomatic patients). Evaluation of the Delise and Sieira scores with different numbers of extra stimuli (1 or 2 vs 3) did not substantially improve the event prediction c-index. CONCLUSIONS: In BrS, classic risk factors such as ECG pattern and previous syncope predict arrhythmic events. The predictive capabilities of the EPS are affected by the number of extra stimuli required to induce ventricular arrhythmias. Scores combining clinical risk factors with EPS help to identify the populations at highest risk, although their predictive abilities remain modest in the general BrS population and in asymptomatic patients.