Reduced desmoplakin immunofluorescence signal in arrhythmogenic cardiomyopathy with epicardial right ventricular outflow tract tachycardia Sabina Rosset,

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Presentation transcript:

Reduced desmoplakin immunofluorescence signal in arrhythmogenic cardiomyopathy with epicardial right ventricular outflow tract tachycardia Sabina Rosset, MD, Argelia Medeiros Domingo, MD, PhD, Angeliki Asimaki, MD, PhD, Denis Graf, MD, Jacques Metzger, MD, Jürg Schwitter, MD, Samuel Rotman, MD, Etienne Pruvot, MD HeartRhythm Case Reports Volume 5, Issue 2, Pages 57-62 (February 2019) DOI: 10.1016/j.hrcr.2018.06.013 Copyright © 2018 Heart Rhythm Society Terms and Conditions

Figure 1 A: Baseline 12-lead electrocardiogram showing normal sinus rhythm with 1.5 mm J-point elevation in V3–V5 in patient 1 and normal sinus rhythm with late R-wave progression in precordial leads and incomplete right bundle branch block in V1 not fulfilling the criterion for an epsilon wave in patient 2. B: Left: Sustained monomorphic ventricular tachycardia (heart rate 214 beats/min) induced during an electrophysiological study in patient 1. Right: Nonsustained ventricular tachycardia (heart rate 230 beats/min) occurring during an exercise stress test in patient 2. C: Progressive occurrence of negative T waves up to V3 over a 3-year follow-up. HeartRhythm Case Reports 2019 5, 57-62DOI: (10.1016/j.hrcr.2018.06.013) Copyright © 2018 Heart Rhythm Society Terms and Conditions

Figure 2 A: Left: Endocardial bipolar map based on a 3-dimensional electroanatomic system in patient 1. Note the presence of a limited area of low-voltage potentials in the anterolateral right ventricular outflow tract (RVOT). Right: Epicardial bipolar map showing an extended area of low-voltage potentials overlying most of the right ventricular (RV) epicardium. Examples of early (a), mid (b), and late (c) late abnormal ventricular activations are shown on the right-hand side. B: Left: Endocardial bipolar map in patient 2. A limited number of fragmented potentials in the anterolateral RVOT are shown as pink dots. Right: Epicardial bipolar map showing an extended area of low-voltage potentials overlying most of the RV epicardium. HeartRhythm Case Reports 2019 5, 57-62DOI: (10.1016/j.hrcr.2018.06.013) Copyright © 2018 Heart Rhythm Society Terms and Conditions

Figure 3 Immunofluorescence staining of intercalated disc proteins in a control subject and in patients 1 and 2. HeartRhythm Case Reports 2019 5, 57-62DOI: (10.1016/j.hrcr.2018.06.013) Copyright © 2018 Heart Rhythm Society Terms and Conditions