Assessing the Malignant Ventricular Arrhythmic Substrate in Patients With Brugada Syndrome

Carlo Pappone, Giuseppe Ciconte, Francesco Manguso, Gabriele Vicedomini, Valerio Mecarocci, Manuel Conti, Luigi Giannelli, Paolo Pozzi, Valeria Borrelli, Lorenzo Menicanti, Zarko Calovic, Giuseppe Della Ratta, Josep Brugada, Vincenzo Santinelli

Research output: Contribution to journalArticlepeer-review


© 2018 The Authors Background: Guidelines recommend the use of implanted cardioverter-defibrillators in patients with Brugada syndrome and induced ventricular tachyarrhythmias, but there is no evidence supporting it. Objectives: This prospective registry study was designed to explore clinical and electrophysiological predictors of malignant ventricular tachyarrhythmia inducibility in Brugada syndrome. Methods: A total of 191 consecutive selected patients with (group 1; n = 88) and without (group 2; n = 103) Brugada syndrome–related symptoms were prospectively enrolled in the registry. Patients underwent electrophysiological study and substrate mapping or ablation before and after ajmaline testing (1 mg/kg/5 min). Results: Overall, before ajmaline testing, 53.4% of patients had ventricular tachyarrhythmia inducibility, which was more frequent in group 1 (65.9%) than in group 2 (42.7%; p <0.001). Regardless of clinical presentation, larger substrates with more fragmented long-duration ventricular potentials were found in patients with inducible arrhythmias than in patients without inducible arrhythmias (p <0.001). One extrastimulus was used in more extensive substrates (median 13 cm2; p <0.001), and ventricular fibrillation was the more frequently induced rhythm (p <0.001). After ajmaline, patients without arrhythmia inducibility had arrhythmia inducibility without a difference in substrate characteristics between the 2 groups. The substrate size was the only independent predictor of inducibility (odds ratio: 4.51; 95% confidence interval: 2.51 to 8.09; p <0.001). A substrate size of 4 cm2 best identified patients with inducible arrhythmias (area under the curve: 0.98; p <0.001). Substrate ablation prevented ventricular tachyarrhythmia reinducibility. Conclusions: In Brugada syndrome dynamic substrate variability represents the pathophysiological basis of lethal ventricular tachyarrhythmias. Substrate size is independently associated with arrhythmia inducibility, and its determination after ajmaline identifies high-risk patients missed by clinical criteria. Substrate ablation is associated with electrocardiogram normalization and not arrhythmia reinducibility. (Epicardial Ablation in Brugada Syndrome [BRUGADA_I]; NCT02641431; Epicardial Ablation in Brugada Syndrome: An Extension Study of 200 BrS Patients; NCT03106701)
Original languageEnglish
Pages (from-to)1631-1646
Number of pages16
JournalJournal of the American College of Cardiology
Issue number15
Publication statusPublished - Apr 17 2018


  • Brugada syndrome
  • catheter ablation
  • mapping
  • programmed ventricular stimulation
  • sudden death
  • ventricular arrhythmias


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