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Natural History of Brugada Syndrome: Insights for Risk Stratification and Management
Abstract
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 V 1 through V 3 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.
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Cited by
Priori, S. G., Blomström-Lundqvist, C., Mazzanti, A., Blom, N., Borggrefe, M., Camm, J., Elliott, P. M., Fitzsimons, D., Hatala, R., Hindricks, G., et al. (2015) · European Heart Journal · doi:10.1093/eurheartj/ehv316 · Cited by 3,893
Self-citationPriori, S. G., Wilde, A. A., Horie, M., Cho, Y., Behr, E. R., Berul, C., Blom, N., Brugada, J., Chiang, C.-E., Huikuri, H., et al. (2013) · Heart Rhythm · doi:10.1016/j.hrthm.2013.05.014 · Cited by 1,942
Self-citationProbst, V., Veltmann, C., Eckardt, L., Meregalli, P. G., Gaita, F., Tan, H. L., Babuty, D., Sacher, F., Giustetto, C., Schulze-Bahr, E., et al. (2010) · Circulation · doi:10.1161/circulationaha.109.887026 · Cited by 847
Self-citationRisk Stratification in Brugada Syndrome
Priori, S. G., Gasparini, M., Napolitano, C., Della Bella, P., Ottonelli, A. G., Sassone, B., Giordano, U., Pappone, C., Mascioli, G., Rossetti, G., et al. (2012) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2011.08.064 · Cited by 581
Self-citationMorita, H., Kusano, K. F., Miura, D., Nagase, S., Nakamura, K., Morita, S. T., Ohe, T., Zipes, D. P., & Wu, J. (2008) · Circulation · doi:10.1161/circulationaha.108.770917 · Cited by 470
Metadata
- Authors
- Priori, S. G., Napolitano, C., Gasparini, M., Pappone, C., Bella, P. D., Giordano, U., Bloise, R., Giustetto, C., De Nardis, R., Grillo, M., Ronchetti, E., Faggiano, G., & Nastoli, J.
- Venue
- Circulation
- Published
- 2002-03-19
- Volume
- 105
- Issue
- 11
- Pages
- 1342-1347
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- OpenAlex
- W2111936199
- Cited by
- 1,034
- References
- 0
- FWCI
- 42.56
- Type
- journal article
- Language
- en
- Open access
- closed
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- CoChat. (2026). Citation check: Natural History of Brugada Syndrome: Insights for Risk Stratification and Management. https://app.cochat.ai/papers/10.1161/hc1102.105288
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- Priori, S. G., Napolitano, C., Gasparini, M., Pappone, C., Bella, P. D., Giordano, U., Bloise, R., Giustetto, C., De Nardis, R., Grillo, M., Ronchetti, E., Faggiano, G., & Nastoli, J. (2002). Natural History of Brugada Syndrome: Insights for Risk Stratification and Management. Circulation. https://doi.org/10.1161/hc1102.105288
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