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  • The appearance of J wave or ER is now recognized

    2019-05-17

    The appearance of J wave or ER is now recognized to predispose to the development of arrhythmogenesis when associated with other cardiac disorders, such as ischemia, JQ1 cost failure, and hypothermia. The J wave might predict prognosis of cardiac events in various heart diseases, and the appearance of a new J wave during acute ischemia seems to be a messenger of VF [240,241]. Family history of sudden death in subjects with ERP has been identified as a risk factor [189,242]. The presence of coexisting Brugada ECG pattern (J waves in V1−V3) or short QT intervals in subjects with ER also suggests a more malignant nature [243,244]. ER is commonly observed in the young, especially in fit and highly trained athletes, with a prevalence ranging up to 40%. In the majority of cases, the ensuing ST segment is rapidly ascending, suggesting that this is a benign ECG manifestation [237,245]. Mahida et al. [246]. recently reported that electrophysiologic (EP) study using programmed stimulation protocols does not enhance risk stratification in ERS.
    Brugada syndrome Numerous studies consistently show that clinical presentation is the strongest predictor of risk in BrS, overshadowing all other risk factors. The risk of recurrent VF among patients presenting with cardiac arrest is considerable: ≈35% at 4 years [247,248], 44% at 7 years [249], and 48% at 10 years [250]. Fortunately, only a minority of patients with BrS (6% in Europe [247] but 18% in Japan [248]) diagnosed today have a history of cardiac arrest. Approximately one-third of contemporary BrS cohorts present with syncope [247]. Their risk of arrhythmic events during follow-up is intermediate: approximately 4 times higher than the risk of asymptomatic patients [49,247,251] but 4 times lower than that of patients diagnosed after cardiac arrest [247]. One explanation for this observation is that the syncope population consists of 2 different groups, one with arrhythmic syncope and bad prognosis, and a second with vagal syncope and good prognosis. Although a detailed clinical history may be of great value in differentiating between these 2 groups, it is not infallible [252]. In reviewing the records of 342 BrS patients, Olde Nordkamp et al. [60]. concluded that arrhythmic and nonarrhythmic syncope can be distinguished by clinical characteristics, including the absence of prodromes and specific triggers. Compared to patients with suspected nonarrhythmic syncope, patients presenting with presumed arrhythmic syncope were more likely to be male (RR 2.1) and to have urinary incontinence (RR 4.6) and were less likely to report prodromes. They also were older at first event (45 vs 20 years), and their syncope was never triggered by hot/crowded surroundings, pain or other emotional stress, sight of blood, or prolonged standing as in the case of nonarrhythmic syncope. During follow-up, all of the spontaneous arrhythmic events occurred in patients who originally presented with presumed arrhythmic syncope; patients with benign syncope had an excellent long-term prognosis. Today asymptomatic patients represent a majority (~63%) of newly diagnosed Brugada patients [247,248]. Their risk of developing symptoms is relatively low (0.5% per year) [247,248]. Unfortunately, for most JQ1 cost the first symptom is cardiac arrest or SCD. Therefore, risk stratification of asymptomatic patients is of utmost importance, and strategies for doing so are discussed later. In cardiac arrest patients or patients with presumed arrhythmic syncope, these strategies are of little benefit because these patients are recognized to be at high risk.
    Update on approaches to therapy for BrS and ERS Figs. 6 and 7 graphically present recommendations for the management of BrS and ERS as modified from the 2013 HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes and the 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of SCD [8,9]. Those recommendations are based on the available literature and on the clinical experience of the Task Force members. As with all such recommendations, they will need to undergo continuous validation in future studies.