title: Heterogeneous distribution of substrates between the endocardium and epicardium promotes ventricular fibrillation in arrhythmogenic right ventricular dysplasia/cardiomyopathy.
abstract: Whether the distribution of scar in arrhythmogenic right ventricular cardiomyopathy (ARVC) plays a role in predicting different types of ventricular arrhythmias is unknown. This study aimed to investigate the prognostic value of scar distribution in patients with ARVC.
  
  
  
  We studied 80 consecutive ARVC patients (46 men, mean age 47 ± 15 years) who underwent an electrophysiological study with ablation. Thirty-four patients receive both endocardial and epicardial mapping. Abnormal endocardial substrates and epicardial substrates were characterized. Three groups were defined according to the epicardial and endocardial scar gradient (<10%: transmural, 10-20%: intermediate, >20%: horizontal, as groups 1, 2, and 3, respectively). Sinus rhythm electrograms underwent a Hilbert-Huang spectral analysis and were displayed as 3D Simultaneous Amplitude Frequency Electrogram Transformation (SAFE-T) maps, which represented the arrhythmogenic potentials. The baseline characteristics were similar between the three groups. Group 3 patients had a higher incidence of fatal ventricular arrhythmias requiring defibrillation and cardiac arrest during the initial presentation despite having fewer premature ventricular complexes. A larger area of arrhythmogenic potentials in the epicardium was observed in patients with horizontal scar. The epicardial-endocardial scar gradient was independently associated with the occurrence of fatal ventricular arrhythmias after a multivariate adjustment. The total, ventricular tachycardia, and VF recurrent rates were higher in Group 3 during 38 ± 21 months of follow-up.
  
  
  
  For ARVC, the epicardial substrate that extended in the horizontal plane rather than transmurally provided the arrhythmogenic substrate for a fatal ventricular arrhythmia circuit.
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.|Department of Medicine, Taipei Veterans General Hospital, Yuanshan Branch, Yilan, Taiwan.
authors.forename: Chin-Yu
authors.initials: CY
authors.lastname: Lin
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Yenn-Jiang
authors.initials: YJ
authors.lastname: Lin
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.|Division of Cardiology, Cardiovascular Center, Taichung Veterans General Hospital, Taiwan.
authors.forename: Cheng-Hung
authors.initials: CH
authors.lastname: Li
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Fa-Po
authors.initials: FP
authors.lastname: Chung
authors.affiliation: Department of Biomedical Sciences and Engineering, Institute of Translational and Interdisciplinary Medicine, National Central University, Taipei, Taiwan.
authors.forename: Men-Tzung
authors.initials: MT
authors.lastname: Lo
authors.affiliation: Department of Biomedical Sciences and Engineering, Institute of Translational and Interdisciplinary Medicine, National Central University, Taipei, Taiwan.
authors.forename: Chen
authors.initials: C
authors.lastname: Lin
authors.affiliation: Department of Biomedical Sciences and Engineering, Institute of Translational and Interdisciplinary Medicine, National Central University, Taipei, Taiwan.
authors.forename: Hsiang-Chih
authors.initials: HC
authors.lastname: Chang
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Shih-Lin
authors.initials: SL
authors.lastname: Chang
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Li-Wei
authors.initials: LW
authors.lastname: Lo
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Yu-Feng
authors.initials: YF
authors.lastname: Hu
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Yao-Ting
authors.initials: YT
authors.lastname: Chang
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Chung-Hsing
authors.initials: CH
authors.lastname: Lin
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Institute of Epidemiology and Preventive Medicine College of Public Health, National Taiwan University, Taipei, Taiwan.
authors.forename: Yun-Yu
authors.initials: YY
authors.lastname: Chen
authors.affiliation: Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Rohit
authors.initials: R
authors.lastname: Walia
authors.affiliation: Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Abigail Louise D
authors.initials: ALD
authors.lastname: Te
authors.affiliation: Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Yamada
authors.affiliation: Division of Cardiology, Cardiovascular Center, Taichung Veterans General Hospital, Taiwan.
authors.forename: Tsu-Juey
authors.initials: TJ
authors.lastname: Wu
authors.affiliation: Faculty of Medicine, Institute of Clinical Medicine, and Cardiovascular Research Center, National Yang-Ming University, Taiwan.|Department of Medicine, Division of Cardiology, Taipei Veterans General Hospital, Taiwan.
authors.forename: Shih-Ann
authors.initials: SA
authors.lastname: Chen
mesh_terms: D000200:Action Potentials; D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D017115:Catheter Ablation; D016757:Death, Sudden, Cardiac / Q000209:etiology; D015716:Electrocardiography, Ambulatory; D022062:Electrophysiologic Techniques, Cardiac; D004699:Endocardium / Q000000981:diagnostic imaging / Q000503:physiopathology*; D005260:Female; D006339:Heart Rate; D006801:Humans; D008297:Male; D008875:Middle Aged; D010496:Pericardium / Q000000981:diagnostic imaging / Q000503:physiopathology*; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery