Natural history of Brugada syndrome: Insights for risk stratification and management

Silvia G. Priori, Carlo Napolitano, Maurizio Gasparini, Carlo Pappone, Paolo Della Bella, Umberto Giordano, Raffaella Bloise, Carla Giustetto, Roberto De Nardis, Massimiliano Grillo, Elena Ronchetti, Giovanna Faggiano, Janni Nastoli

Research output: Contribution to journalArticle

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 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

Original languageEnglish
Pages (from-to)1342-1347
Number of pages6
JournalCirculation
Volume105
Issue number11
DOIs
Publication statusPublished - Mar 19 2002

Keywords

  • Death, sudden
  • Fibrillation
  • Genetics
  • Risk factors
  • Tachyarrhythmias

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

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