Before and after ajmaline, 3D epicardial duration maps displayed in the RVOT large areas of variable size with abnormally prolonged potentials, which contrasted with normal signals in the surrounding areas. The natural history of the BrS, the arrhythmogenic mechanism of the clinical manifestations, and the management of the disease remain elusive, and no single causal factor seems to link all patients with BrS.1–7,9–25 Indeed, in the past decade, with wider diagnosis of the syndrome, its clinical presentation has substantially changed ranging from an initially fatal disorder with documented VF, which may occur late and be the first manifestation of the disease, to several heterogeneous less symptomatic clinical presentations that are often without sudden death or cardiac arrest when initially diagnosed.3 Because it is unclear whether BrS may aggravate over the time, repeated electrophysiology testing is commonly recommended in noninducible patients even after 10 years or before if the patients’ cardiac status changes. Electrogram duration was measured before and after ajmaline in the bipolar signal as the interval between the onset of the first and the offset of the last component of the electrogram, measured at the time scale of 200 mm/s, and expressed as mean bipolar electrogram duration (ms). Color-coded electroanatomical voltage, activation and duration maps were performed and superimposed to cardiac anatomy. Natural history of Brugada syndrome: insights for risk stratification and management. Right, Two examples of electrograms found in the most fragmented and prolonged region (purple area). The high right precordial leads show horizontal and flat ST-segment elevation which disappears during the follow-up (bottom). Below (left-bottom), example of a wide and fragmented potential discovered in the purple area (distal bipolar electrogram 320 ms duration [light blue color]).
Figure 2. These areas were commonly localized in the RVOT, but after ajmaline, they were extended toward the anterior right ventricular free wall in many patients, particularly in those with worst clinical presentation. There is emerging evidence that localization and elimination of abnormal electric activity in the epicardial right ventricular outflow tract may be beneficial in patients with Brugada syndrome. CARTO maps identified epicardial areas of abnormal prolonged electric signals over the RVOT (>75%) extending after ajmaline to RV free wall (Figures 1–5; Figures VI–X in the online-only Data Supplement). Ablation of the epicardial substrate in the right ventricular outflow tract in a patient with Brugada syndrome refusing implantable cardioverter defibrillator therapy. if there be any sorrow like unto my sorrow. ECG changes immediately after ablation and 13 months after the procedure.

Besides the ECG, middle, the epicardial potential duration map (CARTO3) shown in purple color, the area exhibiting long duration potentials (≥200 ms; dimensions 21.5 cm2). Larger abnormal areas and wider abnormal electrograms were found in patients with type 1 ECG pattern than in patients without (Table IV in the online-only Data Supplement). Dallas, TX 75231 We also demonstrated an objective relationship between the degree of type 1 ECG pattern and the extent of the substrate: the wider the abnormal area, the higher the ST-segment elevation and coved-type appearance. Recent case reports and small size studies have reported preliminary data on the presence of an epicardial substrate in symptomatic BrS patients with recurrent VT/VF and implanted ICD, but anatomic and electrophysiological characteristics of the substrate remain undefined and not well established.4–6,15,17,25–27 Because ablation of any arrhythmogenic substrate prevents the development of a later episode of an arrhythmia, the preventive ablation of a potential arrhythmic substrate in patients with BrS would be of benefit in modifying the natural history of the syndrome.
ABL indicates ablation; AP, antero-posterior; INF, inferior; LAO, left anterior oblique; LL, latero-lateral; PA, postero-anterior; RAO, right anterior oblique; RL, right lateral; and SUP, superior. Phenotype abolition by epicardial substrate ablation. Interested. The epicardial access was gained by a percutaneous subxyphoid access to the pericardial space as described by Sosa et al.8 Three-dimensional (3D) RV endocardial and epicardial mapping (CARTO 3, Biosense Webster, Diamond Bar, CA) was performed in all patients during stable sinus rhythm and presence of type I BrS-ECG pattern. Persistent ECG pattern normalization without VF inducibility even after repeated ajmaline challenge suggests that substrate ablation can be considered as a potential therapy for preventing recurrent VT/VF. Insight into the mechanism of Brugada syndrome: epicardial substrate and modification during ajmaline testing. A diagnosis of tubular nephropathy was done by the nephrologists.