Brugada Syndrome or Brugada Electrocardiogram?

We read with great interest the paper by Benito et al. (1) from the
Brugada group. The authors confirm that important differences
exist between men and women regarding the clinical picture and
outcome in patients with Brugada syndrome. In general, in men
the clinical picture appears more severe and the outcome is worse.
Although this may seem presumptuous, we wonder if all subjects
in this study truly had Brugada syndrome, in particular the women.
From the paper, it reads as if the diagnosis “Brugada syndrome” is
synonymous with the presence of a type-1 electrocardiogram
(ECG) (coved-type, either spontaneously or after pharmacologic
provocation with a sodium-channel blocker). It is not mentioned
whether additional clinical features were required for the diagnosis.
According to the consensus statement (2), in addition to a
“Brugada ECG” (type-1 ECG), for Brugada syndrome to be
definitely diagnosed at least 1 of the following clinical features is
required: documented ventricular fibrillation or polymorphic ventricular
tachycardia, a family history of sudden death at
45 years
of age, coved-type ECGs in family members, inducibility of
ventricular tachycardia with programmed electrical stimulation,
syncope, or nocturnal agonal respiration. In other words, a Brugada
ECG is not sufficient to diagnose Brugada syndrome, and
this is a very important point both in clinical practice and in
scientific studies. When applying the above criteria to the study by
Benito et al. (1), some uncertainty remains. According to Table 2
in their paper (1), in women syncope was present “only” in 15%,
aborted sudden cardiac death in 1%, a history of sudden cardiac
death in 45%, and programmed electrical stimulation was performed
in 81% with inducibility in 12%. If anything, these figures
do not add up to 100%. Of note, there was also a difference in the
baseline ECG; as many as 62% of the women had a normal or
type-3 ECG as opposed to only 25% of the men. Obviously, in
subjects in whom the diagnosis of Brugada syndrome is not firmly
established, a mild clinical picture and a good outcome can be
expected.

*Maarten P. van den Berg, MD, PhD
Rudolf A. de Boer, MD, PhD
J. Peter van Tintelen, MD, PhD