Quinidine for Brugada syndrome: Panacea or poison?

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Presentation transcript:

Quinidine for Brugada syndrome: Panacea or poison? Jo-Jo Hai, MBBS, Chun-Ka Wong, MBBS, Pak-Hei Chan, MBBS, Hung-Fat Tse, MD, PhD, Tak-Cheung Yung, MBBS, Chung-Wah Siu, MD  HeartRhythm Case Reports  Volume 2, Issue 6, Pages 486-490 (November 2016) DOI: 10.1016/j.hrcr.2016.07.002 Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 1 A: Standard 12-lead electrocardiogram (ECG) showing partial right bundle branch block with a coved ST-segment elevation and negative T-wave deflections in leads V1 and V2, compatible with type I Brugada pattern; the corrected QT (QTc) interval was 493 msec. B: The stored ECGs showed an episode of ventricular fibrillation/tachycardia, triggering an implantable cardioverter-defibrillator shock. C: Standard 12-lead ECG showing frequent premature ventricular complexes of right ventricular outflow tract origin. D: In-hospital telemetry recording showing an episode of torsades de pointes initiated by a long–short sequence of ventricular bigeminy (asterisk). HeartRhythm Case Reports 2016 2, 486-490DOI: (10.1016/j.hrcr.2016.07.002) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 2 The sequence chromatogram of the 2 mutations: R1192Q (A) and E1784K (B) in SCN5A, the gene encoding Nav1.5, the α-subunit of the cardiac sodium channel. C: Schematic representation of the primary structure of α-subunit of the cardiac sodium channel with locations of 2 SCN5A mutations. HeartRhythm Case Reports 2016 2, 486-490DOI: (10.1016/j.hrcr.2016.07.002) Copyright © 2016 Heart Rhythm Society Terms and Conditions

Figure 3 A: The effects of mexiletine (150 mg) on the corrected QT interval during atrial paced rate of 70 beats per minute. B: Premature ventricular complexes ablated sequentially at the anterior right ventricular outflow tract, subpulmonary valvular region, and anterior right ventricular wall. Purkinje potentials were seen at the final ablation site. HeartRhythm Case Reports 2016 2, 486-490DOI: (10.1016/j.hrcr.2016.07.002) Copyright © 2016 Heart Rhythm Society Terms and Conditions