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Background: We aimed to develop a risk score (LGE Based Prediction of SCD Risk in Nonischemic Dilated Cardiomyopathy [ESTIMATED]) based on late gadolinium enhancement (LGE) cardiac magnetic resonance to predict sudden cardiac death (SCD) in patients with nonischemic dilated cardiomyopathy (NIDCM) and left ventricular ejection fraction ≤ 35%., Methods: We recruited 395 consecutive middle-aged patients with NIDCM and performed 3-year follow-up for SCD events. The score was developed and verified in 295 primary prevention patients, and the predictive value was confirmed by comparing the SCD events between the high-risk patients stratified by the score and 100 secondary prevention patients., Results: The ESTIMATED score (constructed by the LGE extent > 14%, syncope, atrial flutter/fibrillation, nonsustained ventricular tachycardia, advanced atrioventricular block, and age ≤ 20 or > 50 years) showed good calibrations for SCD prediction in the derivation (C-statistic: 0.80, 95% confidence interval: 0.74-0.86) and validation set (C-statistic: 0.80, 95% confidence interval: 0.71-0.87). By the score, 20.3% of primary prevention patients were categorized as high risk (≥ 3 points), 28.1% as intermediate risk (2 points), and 51.6% as low risk (0-1 points) for 3-year SCD events (45.9% vs 20.1% vs 5.1%, P < 0.0001). The 3-year SCD events were also well in agreement with the score stratification in patients without implantable cardioverter-defibrillator. High-risk primary prevention patients selected by the score in the derivation and validation sets had 3-year SCD events comparable with that in secondary prevention patients (47.6% vs 40.6% vs 38.7%, P = 0.81)., Conclusions: Our study derived and validated an LGE-based (ESTIMATED) risk score providing refined SCD prediction. The score may help to identify candidates for primary prevention implantable cardioverter-defibrillator in patients with NIDCM., (Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Catheter ablation in the right ventricular outflow tract (RVOT) may modify the electrophysiologic substrate for recurrent ventricular tachycardia/ventricular fibrillation (VT/VF) in patients with Brugada syndrome (BrS)., Objective: The purpose of this study was to investigate the mechanism and arrhythmogenic substrate of VT/VF and to evaluate the long-term outcomes of catheter ablation in patients with BrS., Methods: Eleven consecutive patients with BrS referred to 2 academic medical centers underwent combined epicardial-endocardial electroanatomic mapping. Catheter ablation was performed in regions of localized conduction slowing. Transmural dispersion of late activation was calculated as the difference between the latest activation between epicardium and endocardium, and low-voltage areas were analyzed., Results: Eleven patients met diagnostic criteria for BrS (spontaneous type 1, n = 9; Na channel provocation = 2). All patients were found to have a localized region in the anterior epicardial RVOT with conduction slowing evidenced by prolonged electrogram duration (78.79 ± 19.87 ms vs 58.93 ± 10.11 ms in epicardial right ventricle, and 59.87 ± 12.61 ms in endocardial RVOT, P <.005, respectively) with variable low voltage (0.97 ± 0.48 mV; median scar area 19.8 ± 25.9 cm 2 ). Epicardial ablation resulted in normalization of spontaneous type 1 Brugada ECG pattern in all patients, and 73% were free from VT/VF at 25 ± 11 months., Conclusion: Prolonged electrograms localized to epicardial RVOT with variable low voltage were identified in all patients with BrS. J-point and ST-segment elevation correlated with greater transmural dispersion of late activation and was independent of total low-voltage area. Despite normalization of spontaneous type 1 pattern in all patients after ablation, recurrence was still observed, suggesting the implantable cardioverter-defibrillator as the cornerstone therapy for BrS., (Copyright © 2016 Heart Rhythm Society. All rights reserved.)