BACKGROUND: Treatment of patients with Brugada syndrome is complicated by the incomplete information on the natural history of the disease related to the small number of cases reported. Furthermore, the value of programmed electrical stimulation (PES) for risk stratification is highly debated. The objective of this study was to search for novel parameters to identify patients at risk of sudden death. METHODS AND RESULTS: Clinical data were collected for 200 patients (152 men, 48 women; age, 41+/-18 years) and stored in a dedicated database. Genetic analysis was performed, and mutations on the SCN5A gene were identified in 28 of 130 probands and in 56 of 121 family members. The life-table method of Kaplan-Meier used to define the cardiac arrest-free interval in patients undergoing PES failed to demonstrate an association between PES inducibility and spontaneous occurrence of ventricular fibrillation. Multivariate Cox regression analysis showed that after adjusting for sex, family history of sudden death, and SCN5A mutations, the combined presence of a spontaneous ST-segment elevation in leads V1 through V3 and the history of syncope identifies subjects at risk of cardiac arrest (HR, 6.4; 95% CI, 1.9 to 21; P<0.002). CONCLUSIONS: The information on the natural history of patients obtained in this study allowed elaboration of a risk-stratification scheme to quantify the risk for sudden cardiac death and to target the use of the implantable cardioverter-defibrillator.
BACKGROUND: Treatment of patients with Brugada syndrome is complicated by the incomplete information on the natural history of the disease related to the small number of cases reported. Furthermore, the value of programmed electrical stimulation (PES) for risk stratification is highly debated. The objective of this study was to search for novel parameters to identify patients at risk of sudden death. METHODS AND RESULTS: Clinical data were collected for 200 patients (152 men, 48 women; age, 41+/-18 years) and stored in a dedicated database. Genetic analysis was performed, and mutations on the SCN5A gene were identified in 28 of 130 probands and in 56 of 121 family members. The life-table method of Kaplan-Meier used to define the cardiac arrest-free interval in patients undergoing PES failed to demonstrate an association between PES inducibility and spontaneous occurrence of ventricular fibrillation. Multivariate Cox regression analysis showed that after adjusting for sex, family history of sudden death, and SCN5A mutations, the combined presence of a spontaneous ST-segment elevation in leads V1 through V3 and the history of syncope identifies subjects at risk of cardiac arrest (HR, 6.4; 95% CI, 1.9 to 21; P<0.002). CONCLUSIONS: The information on the natural history of patients obtained in this study allowed elaboration of a risk-stratification scheme to quantify the risk for sudden cardiac death and to target the use of the implantable cardioverter-defibrillator.
Authors: Mackenzi Mbai; Sridharan Rajamani; Brian P Delisle; Blake D Anson; Corey Anderson; Jonathan C Makielski; Craig T January Journal: Curr Cardiol Rep Date: 2002-09 Impact factor: 2.931
Authors: Christian Wolpert; Constanze Echternach; Christian Veltmann; Charles Antzelevitch; George P Thomas; Susanne Spehl; Florian Streitner; Juergen Kuschyk; Rainer Schimpf; Karl K Haase; Martin Borggrefe Journal: Heart Rhythm Date: 2005-03 Impact factor: 6.343
Authors: Dan Hu; Hector Barajas-Martínez; Andre Terzic; Sungjo Park; Ryan Pfeiffer; Elena Burashnikov; Yuesheng Wu; Martin Borggrefe; Christian Veltmann; Rainer Schimpf; John J Cai; Gi-Byong Nam; Pramod Deshmukh; Melvin Scheinman; Mark Preminger; Jonathan Steinberg; Angélica López-Izquierdo; Daniela Ponce-Balbuena; Christian Wolpert; Michel Haïssaguerre; José Antonio Sánchez-Chapula; Charles Antzelevitch Journal: Int J Cardiol Date: 2014-01-04 Impact factor: 4.164