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  • Education and lifestyle changes for


    Education and lifestyle changes for the prevention of arrhythmias are critical in BrS. Patients should be informed of the various modulators and precipitating factors that could cause malignant arrhythmias. A prominent S-wave in Lead I has recently been identified as an ECG marker of BrS [298A]. Fever should be treated aggressively with antipyretics, and contraindicated substances should be avoided (see [89]. Referral for ECG is recommended during high fever. Family members may be referred for cardiopulmonary resuscitation training and advised to consider purchasing an automatic external defibrillator for home use. Because malignant ventricular arrhythmias are infrequent in asymptomatic patients with BrS [247] or ERP [44] and usually unrelated to physical activity, the presence of these patterns does not contraindicate participation in sports. It is noteworthy, however, that the Brugada pattern is accentuated immediately after exercise, presumably because of an increase in vagal tone [292,299,300]. In reviewing 98 case of BrS studies dealing with exercise, Masrur et al. [299]. concluded that there are insufficient data on the risks of exercise in BrS to make recommendations for exercise.
    Implantable cardioverter-defibrillator The only proven effective therapeutic strategy for the prevention of SCD in high-risk BrS and ERS patients is an ICD [301,302]. It is important to recognize that ICDs are associated with complications, especially in young active individuals [249,303]. At 10 years postimplantation, the rates of inappropriate shock and lead failure are 37% and 29%, respectively. Remote monitoring can identify lead failure and prevent inappropriate shocks [304]. Subcutaneous ICDs are thought to represent the future for this indication because they BEZ 235 are expected to be associated with fewer complications over a lifetime [305]. Implantation of an ICD is first-line therapy for JWS patients presenting with aborted SCD or documented VT/VF with or without syncope (Class I recommendation) [301,306]. ICDs can be useful (Class IIa) in symptomatic BrS patients with type 1 pattern, in whom syncope was likely caused by VT/VF. The HRS/EHRA/APHRS expert consensus states that the ICD may be considered (Class IIb) in asymptomatic patients with inducible VF during programmed electrical stimulation (PES) [8]. Some studies suggest that the predictive value of EP studies may be improved by limiting the PES protocol to 2 extrastimuli [50,276], but that observation is not supported by other studies [49,307]. Similarly, some studies advocate that PES should be limited to the RVA and credit this limited PES strategy for a very high positive predictive value found is some series [275]. Again, that observation is not confirmed by other studies [276]. The current Task Force proposes that ICDs are reasonable (Class IIa) in symptomatic BrS patients with type 1 pattern but that implantation be considered on a case-by-case basis by an electrophysiologist experienced in BrS, taking into consideration age, gender, clinical presentation, ECG characteristics (QRS fragmentation, Jp amplitude), and patient preference. The current Task Force also proposes that EP study may be considered in asymptomatic individuals with spontaneous type 1 Brugada pattern. If VT/VF is inducible, an ICD should be considered [7]. More recent studies argue in favor of using ≤2 extrastimuli to induce VT/VF [50,276]. ICDs are not indicated in asymptomatic patients without any of these characteristics. At present, there is no clear role for PES in patients with ERS.
    Pacemaker therapy Arrhythmic events and SCD in both BrS and ERS generally occur during sleep or at rest and are associated with slow heart rates. These observations notwithstanding, a potential therapeutic role for cardiac pacing remains largely unexplored [308]. A few case reports are available [309,310].
    RFA therapy Nademanee et al. [129]. showed that RFA of epicardial sites displaying late potentials and fractionated bipolar electrograms in the RVOT of BrS patients can significantly reduce arrhythmia vulnerability and the ECG manifestation of the disease. Ablation at these sites was reported to render VT/VF noninducible and to normalize the Brugada ECG pattern in the vast majority of patients over a period of weeks or months. Long-term follow-up (20–6 months) showed no recurrent VT/VF, with only 1 patient on medical therapy with amiodarone. Case reports in support of these effects have been published [311]. Additional evidence in support of the effectiveness of epicardial substrate ablation was provided by Sacher et al. [130]. and Shah et al. [312].