The Brugada Numbers
To the Editor:
In a Consensus Report on proposed diagnostic criteria for the Brugada syndrome, Wilde et al1 report an incidence “ranging between 5 and 66 per 10 000.” With a world population of 6 157 000 000,2 that would mean between 3 000 000 and 40 000 000 have the Brugada syndrome, similar in scope to the estimated 36 100 000 infected with HIV.2 This cannot be. More likely, the quoted numbers reflect the prevalence of the Brugada electrocardiographic pattern (the “Brugada sign”).3 Herein lies the dilemma. As the authors and others have clearly stated, the Brugada sign should not be equated with the Brugada syndrome.1,4 How then do we judiciously screen millions of individuals with the Brugada sign, most of whom are not at risk for sudden death, to find the small percentage with the Brugada syndrome who are at risk of sudden death and in need of a defibrillator? The report by the Arrhythmia Working Group1 as well as other recent publications5 are “works in progress” to help answer that question.
We thank Drs Littmann and Monroe for their letter to the editor. The numbers stemming from the calculations presented by Drs Littmann and Monroe are perhaps not as dramatic as suggested, in that the incidence of 5 per 10 000 is limited to those regions of the world where the Brugada syndrome is endemic—parts of Southeast Asia and Japan. The incidence of 66 per 10 000 quoted in our consensus report1 is limited to a small region in Japan. Moreover, it is important to recognize that the actual prevalence of the syndrome is a sensitive function of the criteria applied in the individual studies,2 which is one of the main reasons for the consensus conference and report. With less than a decade since the identification of the Brugada syndrome as a distinct clinical entity, it would be safe to conclude that data on its worldwide incidence remain out of reach. We take no issue with the other excellent points made in the letter and consider the question of how one might go about screening for those at risk for sudden death as rhetorical in that this is the subject of the consensus document.
Wilde AAM, Antzelevitch C, Borggrefe M, et al. Diagnostic criteria for the Brugada syndrome: a consensus report. Circulation. 2002; 106: 2514–2519.
Remme CA, Wever EFD, Wilde AAM, et al. Diagnosis and long-term follow-up of Brugada syndrome in patients with idiopathic ventricular fibrillation. Eur Heart J. 2001; 22: 400–409.