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br Risk stratification in BrS Many risk
Risk stratification in BrS
Many risk markers have been reported in patients with high-risk BrS. Clinical markers include family history of sudden death at a young age (<40–50 years), syncope with nonprodrome episodes [32], and episodes of paroxysmal atrial fibrillation (PAF) [13,14]. The ECG markers of repolarization abnormalities (increasing transmural dispersion or repolarization instability) are spontaneous type 1 ECG [2], dispersion between the peak and end of the T wave (T peak-end), T wave alternans (TWA) after administration of a sodium channel blocker [33], ST elevation after a large meal [34], abnormal restitution properties [35], effective refractory cotransport in the right ventricle (<200ms) [36], ST-segment elevation after exercise [37], QT prolongation or shortening, and early repolarization pattern in the inferolateral leads [38]. The ECG markers of depolarization abnormalities (exacerbating repolarization abnormality) include prolonged PQ/QRS/HV intervals [1], existence of f-QRS (Fig. 5) [6], and positive late potentials [39]. VF inducibility by programmed electrical stimulation may also predict outcomes, although its predictive accuracy remains controversial. These markers only suggest the presence of underlying electrophysiological abnormality/instability in BrS patients. However, the precise mechanism in each BrS patient is nonuniform, and spontaneous changes of these markers are frequently observed. Therefore, repeated evaluation of these markers should be performed individually.
Treatment
Asymptomatic patients
Risk stratification and the optimal therapeutic approach for asymptomatic BrS patients remain controversial. Initially, Brugada et al. showed that asymptomatic patients in whom VF was induced during an electrophysiological study were at high risk of sudden death [29]. Japanese guidelines in 2011 also recommended the usefulness of electrophysiological studies in BrS (Table 2) [54]. However, recent studies, including those conducted in Japan, have shown that the prevalence of asymptomatic BrS is quite low (around 0.5% per year) and have questioned the usefulness of electrophysiological studies for risk stratification in these patients [8,36,38,55–57]. Recently, Priori et al. reported that shortening of the ventricular refractory period at the right ventricle (<200ms) and f-QRS were related to cardiac events (Fig. 6) [36]. However, it is still not clear how useful these markers are for identifying asymptomatic BrS patients. Therefore, the establishment of markers for the detection of high-risk and/or low-risk asymptomatic BrS patients is an important issue.
Conclusion
Conflict of interest
Introduction
Brugada syndrome (BS) is characterized by ST-segment elevation in the right precordial leads and is associated with sudden cardiac death secondary to polymorphic ventricular tachycardia (PVT)/ventricular fibrillation (VF) in the absence of structural heart disease [1–3]. Various worldwide BS studies have been conducted over the past 20 years, and significant body of knowledge has been obtained regarding this syndrome (i.e., epidemiology, electrophysiology, pathophysiology, and genetics). However, the relationship between some concomitant clinical abnormalities and BS is poorly understood. Herein, we review recent advances in the understanding of 2 clinical features, vasospastic angina (VSA) and neurally mediated syncope (NMS), which seem to be closely associated with BS.
Vasospastic angina and Brugada syndrome
Neurally mediated syncope and Brugada syndrome
Conclusions
Conflict of interest
Introduction
Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome that is associated with ST segment elevation on right precordial leads and a relatively high incidence of sudden cardiac death. BrS is characterized by an electrocardiogram (ECG) pattern manifesting as prominent J waves, giving the appearance of an ST segment elevation in the right precordial leads on an ECG. Diagnostic criteria require the presence of a coved-type ST segment elevation with a J point elevation ≥2mV [1].