Depolarization versus repolarization abnormality underlying inferolateral J-wave syndromes: New concepts in sudden cardiac death with apparently normal.

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Depolarization versus repolarization abnormality underlying inferolateral J-wave syndromes: New concepts in sudden cardiac death with apparently normal hearts Michel Haïssaguerre, MD, Koonlawee Nademanee, MD, Mélèze Hocini, MD, Ghassen Cheniti, MD, Josselin Duchateau, MD, Antonio Frontera, MD, Frédéric Sacher, MD, Nicolas Derval, MD, Arnaud Denis, MD, Thomas Pambrun, MD, Rémi Dubois, PhD, Pierre Jaïs, MD, David Benoist, PhD, Richard D. Walton, PhD, Akihiko Nogami, MD, Ruben Coronel, MD, PhD, Mark Potse, PhD, Olivier Bernus, PhD Heart Rhythm DOI: 10.1016/j.hrthm.2018.10.040 Copyright © 2018 The Authors Terms and Conditions

Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 1 A: Electrocardiographic (ECG) variations in inferolateral J waves. Left: Valsalva or strong inspiration maneuver producing J-wave amplification. Middle: Cycle length prolongation associated with either unchanged pattern or amplification. Right: Negative J waves in inferior leads and positive J waves in lateral leads. Note the increase of J wave after the pause (arrow). B: Hierarchical view of ECG and clinical risk factors. LQT = long QT; SCD = sudden cardiac death; SHD = structural heart disease; VPB = ventricular premature beat. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 2 Inferolateral J-wave syndrome due to abnormal depolarization. Top middle maps show bipolar electrogram voltage (purple indicating voltage >1 mV) with low voltage in inferior right ventricle. Bottom middle maps show activation mapping, with blue indicating the latest activated regions, here in the inferobasal right ventricle. Right: Fragmented electrograms (Egms) preceding and coincident with the J wave (white contour) in bipolar and unipolar (arrows) mode. Left: The 12 lead-ECG in a 19 year- old man who survived VF. ECG = electrocardiogram. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 3 Inferolateral J-wave syndrome due to abnormal depolarization. The maps (middle) show activation mapping, with blue indicating the latest activated regions, here the inferobasal right and left ventricles. Right: Low-voltage fragmented electrograms (Egms) coincident with J wave are only present in the inferior right ventricle (white contour) compared with Egms in the inferior left ventricle. Left: The 12 lead-ECG in a 31 year- old man who survived VF. ECG = electrocardiogram. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 4 Inferolateral J-wave syndrome due to early repolarization. Top middle maps show bipolar electrogram voltage without evidence of low-voltage area. Bottom middle maps shows the latest activated regions (blue) in the inferobasal and laterobasal right ventricle. Right: There are no late depolarization electrograms coincident to the J wave but slow early repolarization potentials (arrows), which are present in the apical region (white-dotted area). Note that the J wave is small on lead II (right) and underestimates the extent of early repolarization recorded by epicardial mapping. Left: A 6 lead- ECG in a 15 year-old girl with recurrent VF. ECG = electrocardiogram. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 5 Another case of early repolarization. Twelve-lead ECGs (left) show a global J-wave pattern. There are no late depolarization bipolar electrograms coincident with the J wave (between the red lines), but early repolarization potentials (arrows) are recorded diffusely in the inferior left ventricle (right). Ant = anterior; ECG = electrocardiogram; inf = inferior; LV = left ventricle. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 6 Typical location of ventricular fibrillation (VF) driver regions. The locations of reentries are shown in red. They are predominantly located in the right ventricle in late depolarization J waves (left) vs the inferior septum in early repolarization (right). Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions

Figure 7 Spectrum of arrhythmogenic diseases leading to sudden cardiac death in apparently normal hearts and proposal of a mechanistic classification based on the primary pathogenesis. IVF = idiopathic ventricular fibrillation; VT = ventricular tachycardia. Heart Rhythm DOI: (10.1016/j.hrthm.2018.10.040) Copyright © 2018 The Authors Terms and Conditions