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Temporal progression and utility of the Shanghai Score in Brugada Syndrome risk stratification
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Advisor(s)
Abstract(s)
Introdução: Apesar do conhecimento sobre a Síndrome de Brugada (SBr) ter evoluído nas últimas três décadas, abrangendo a sua origem genética, a sua fisiopatologia e as suas características clínicas, persistem áreas de incerteza, principalmente quanto à estratificação de risco destes doentes. O Score de Shanghai foi desenvolvido como ferramenta para padronizar o diagnóstico da SBr.
Objetivos: Avaliar a progressão temporal do Score de Shanghai numa coorte de doentes com SBr e explorar sua utilidade como estratificador de prognóstico.
Métodos: Realizamos um estudo prospetivo num único centro hospitalar de doentes com SBr. O Score de Shanghai foi inicialmente calculado no momento do diagnóstico e posteriormente recalculado anualmente ao longo do tempo. Foi considerada para análise a última determinação do Shangai Score anterior à ocorrência de eventos arrítmicos malignos. O outcome principal considerado foi a ocorrência de eventos arrítmicos malignos, definidos como morte súbita, fibrilhação ventricular ou taquicardia ventricular durante o seguimento. Foram utilizadas as análises de sobrevida de Kaplan-Meier para avaliar a associação do outcome com o Score de Shanghai inicial e, também, da última avaliação anual pré-evento.
Resultados: Foi caracterizada uma população constituída por 163 doentes (48 ± 13 anos; 68,7% do sexo masculino), 74,7% assintomáticos e 63,8% com padrão tipo 1 induzido por prova farmacológica. No total, 18,5% dos doentes apresentavam história de síncope, potencialmente arrítmica em 6,8%, e 4,3% tinham história de paragem cardiorrespiratória abortada. Durante um seguimento médio de 57 meses, a mediana do score de Shanghai aumentou significativamente de 3,0 para 3,5 (P < 0,001). O score de Shanghai associou-se significativamente ao risco arrítmico a longo prazo, tendo o mesmo sido determinado na avaliação inicial [Hazard ratio (HR): 1,93; intervalo de confiança (IC) 95%: 1,18 – 3,15; P = 0,009], ou na última consulta (HR: 1,88; 95% CI: 1,26 – 2,79; P = 0,002). A sobrevivência livre de eventos arrítmicos malignos foi significativamente pior entre os doentes com diagnóstico definitivo de SBr,particularmente baseado no score de Shanghai determinado na última consulta (P-valor log rank Mantel-Cox: 0.049).
Conclusões: Este estudo confirma a utilidade do score de Shanghai na estratificação prognóstica dos doentes com SBr. Além disso, demonstrou o caráter dinâmico da SBr, traduzido na modificação da sua expressão fenotípica ao longo do tempo, repercutido no aumento do score de Shanghai. Por isso, realça a importância da reavaliação periódica do risco ao longo do tempo.
Introduction: Over the past three decades, the understanding of Brugada Syndrome (BrS) has expanded, encompassing its genetic background, pathophysiology, and clinical characteristics. However, uncertainty persists concerning the risk stratification for these patients. The Shanghai score was developed as a tool to standardize the diagnosis of BrS. Objectives: To evaluate the temporal progression of the Shanghai score in a cohort of BrS patients and to explore its usefulness for prognostic stratification. Methods: We conducted a prospective single-center study of BrS patients. The Shanghai Score was initially calculated at the time of diagnosis and subsequently recalculated annually over time. The latest determination of the Shangai Score prior to the occurrence of events was considered for analysis. The primary endpoint was the occurrence of malignant arrhythmic events (MAE), defined as sudden cardiac death (SCD), ventricular fibrillation (VF), or ventricular tachycardia (VT) during follow-up. Kaplan-Meier survival analyses were used to assess the association of the Shanghai Score both at baseline and at the last pre-event annual evaluation with the outcomes. Results: The population consisted of 163 patients (48 ± 13 years; 68.7% male), 74.7% asymptomatic and 63.8% with a type 1 pattern induced in provocative pharmacological testing. In total, 18.5% of patients had a history of syncope, potentially arrhythmic in 6.8%, and 4.3% had survived a previous cardiac arrest. During a median follow-up of 57 months, the median Shanghai Score increased significantly from 3.0 to 3.5 (P < 0.001). The Shanghai score was significantly associated with the long-term arrhythmic risk, both at the initial assessment (Hazard ratio (HR): 1.93; 95% confidence interval (CI): 1.18 – 3.15; P = 0.009) and at the last follow-up (HR: 1.88; 95% CI: 1.26 – 2.79; P = 0.002). The event-free survival of malignant arrhythmic events was significantly worse among patients with a definite diagnosis of SBr, particularly based on the Shanghai score determined at the last follow-up (log-rank Mantel-Cox P-value: 0.049) Conclusions: Our study indicates that the Shanghai score holds promise for prognostic stratification in BrS patients. Furthermore, it underscores the significance of periodically re-evaluating the score over time.
Introduction: Over the past three decades, the understanding of Brugada Syndrome (BrS) has expanded, encompassing its genetic background, pathophysiology, and clinical characteristics. However, uncertainty persists concerning the risk stratification for these patients. The Shanghai score was developed as a tool to standardize the diagnosis of BrS. Objectives: To evaluate the temporal progression of the Shanghai score in a cohort of BrS patients and to explore its usefulness for prognostic stratification. Methods: We conducted a prospective single-center study of BrS patients. The Shanghai Score was initially calculated at the time of diagnosis and subsequently recalculated annually over time. The latest determination of the Shangai Score prior to the occurrence of events was considered for analysis. The primary endpoint was the occurrence of malignant arrhythmic events (MAE), defined as sudden cardiac death (SCD), ventricular fibrillation (VF), or ventricular tachycardia (VT) during follow-up. Kaplan-Meier survival analyses were used to assess the association of the Shanghai Score both at baseline and at the last pre-event annual evaluation with the outcomes. Results: The population consisted of 163 patients (48 ± 13 years; 68.7% male), 74.7% asymptomatic and 63.8% with a type 1 pattern induced in provocative pharmacological testing. In total, 18.5% of patients had a history of syncope, potentially arrhythmic in 6.8%, and 4.3% had survived a previous cardiac arrest. During a median follow-up of 57 months, the median Shanghai Score increased significantly from 3.0 to 3.5 (P < 0.001). The Shanghai score was significantly associated with the long-term arrhythmic risk, both at the initial assessment (Hazard ratio (HR): 1.93; 95% confidence interval (CI): 1.18 – 3.15; P = 0.009) and at the last follow-up (HR: 1.88; 95% CI: 1.26 – 2.79; P = 0.002). The event-free survival of malignant arrhythmic events was significantly worse among patients with a definite diagnosis of SBr, particularly based on the Shanghai score determined at the last follow-up (log-rank Mantel-Cox P-value: 0.049) Conclusions: Our study indicates that the Shanghai score holds promise for prognostic stratification in BrS patients. Furthermore, it underscores the significance of periodically re-evaluating the score over time.
Description
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2024
Keywords
Síndrome de Brugada Score de Shanghai Estratificação de risco Prognóstico Cardiologia