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    • No evidence has been published from Japan on whether or

    2019-04-23

    No evidence has been published from Japan on whether or not patients with more than two risk factors have a significantly lower rate of freedom from events than those with less than one risk factor. However, a group from Italy investigated the risk nae inhibitor in individuals with type 1 ECG for the primary prevention of sudden death in 2011, [53] and 320 patients (258 male patients; median age, 43 years) with type 1 ECG were enrolled. No patients had any previous cardiac arrests, and 54% of the patients had spontaneous and 46% had drug-induced type 1 ECG. One-third of the patients had syncope, and 2/3 were asymptomatic. A total of 245 patients underwent an EP study and 110 patients received an ICD. During the follow-up (median length, 40 months), 17 patients (5.3%) had major arrhythmic events (14 resuscitated VF and three sudden cardiac deaths). Both spontaneous type 1 ECG and syncope significantly increased the risk (2.6 and 3.0% event rate per year vs. 0.4 and 0.8%, respectively). Major arrhythmic events occurred in 14% of the subjects with a positive EP study, in no subjects with a negative EP study, and in 5.3% of subjects without an EP study. All major arrhythmic events occurred in subjects who had at least two potential risk factors (syncope, family history, and positive EP study). Among these patients, those with a spontaneous type 1 ECG had a 30% event rate. Thus, the investigators concluded that a multiparametric approach (including assessment for syncope history, family history, and EP study) helped identify the populations at the highest risk and that the subjects at the highest risk were those with a spontaneous type 1 ECG and at least two risk factors (syncope, family history, and positive EP study) and that others were at low-risk [53]. This evidence strongly supports that the Japanese Guidelines 2007 [51] on Brugada syndrome are correct in terms of the risk stratification in asymptomatic Brugada patients. The recent Japanese Guidelines 2011 [52] were proposed with the same basic concept as the previous Guidelines 2007 [51].
    Summary
    Conflict of interest
    Introduction Two decades ago, Pedro and Joseph Brugada described a group of 8 patients with a normal heart who suffered ventricular fibrillation (VF) or sudden cardiac death and had an abnormal electrocardiogram (ECG) of coved type ST elevation over the right precordial leads [1]. It was acknowledged instantly as the Brugada syndrome (BrS), which has also been linked with sudden unexpected death syndrome (SUDS) that usually occurs at night in young Southeast Asian men with a normal heart [2,3]. BrS now is well recognized as a common autosomal dominant inherited arrhythmia disorder with gene mutations that are predominantly confined to the SCN5A gene, which encodes for α-subunit of sodium channel, causing loss of INa[3–6]. Treatment of BrS patients is a significant challenge because there are limited treatment options, and an implantable cardioverter–defibrillator (ICD) is the only choice for high-risk BrS patients (i.e., those who had aborted sudden cardiac death or had previous VF episodes) [7–10]. Unfortunately, even though ICDs are effective at reverting VF episodes to sinus rhythm, they do not prevent VF occurrence. Thus, when some BrS patients experience frequent recurrences of VF episodes necessitating frequent ICD discharges, the so-called “electrical storm” (ES), physicians face the daunting task of suppressing such VF episodes. Fortunately, in recent years, we have learned a great deal more about the syndrome and have therapeutic advances that enable us to treat BrS patients with ES much more effectively than a decade ago. Our review herewith will discuss these advances and how to treat ES in BrS patients most effectively.
    Definition and incidence of electrical storm ES is defined as three or more episodes of VF per day recorded by the ICD interrogation or documented in the recording in the intensive care unit (ICU) (Fig. 1). The true incidence of ES in the BrS is difficult to ascertain. Thus far, most of the reports of ES have been case reports or studies with a very small number of patients. The incidence of ES in our BrS population in Thailand is 5–7% in symptomatic patients, but none in our asymptomatic BrS population.