14 | Chapter 1 Table 1. 2010 Task Force criteria proposed by Marcus et al13 (continued) Depolarization abnormalities (ECG) Major Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 -V3) Minor Late potentials by SA-ECG in ≥ 1 of 3 parameters in the absence of a QRS duration of 110 ms on the standard ECG • Filtered QRS duration (fQRS) ≥ 114 ms • Duration of terminal QRS < 40 µV (low-amplitude signal duration) ≥ 38 ms • Root-mean-square voltage of terminal 40 ms ≤ 20 µV Terminal activation duration of QRS ≥ 55 ms measured from the nadir of the S wave to the end of the QRS, including R’, in V1, V2, or V3, in the absence of complete RBBB Ventricular arrhythmias Major Nonsustained or sustained ventricular tachycardia of LBBB morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL) Minor Nonsustained or sustained ventricular tachycardia of RV outflow configuration, LBBB morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis >500 premature ventricular complexes per 24 hours (Holter) Family history Major ARVC definite diagnosis confirmed in a first-degree relative who meets current 2010 Task Force criteria ARVC definite diagnosis confirmed pathologically at autopsy or surgery in a firstdegree relative Identification of a pathogenic ARVC related mutation categorized as associated or probably associated with ARVC. Plakoglobin (JUP), Desmoplakin (DSP), Plakophilin-2 (PKP2), Desmoglein-2 (DSG2), Desmocollin-2 (DSC2), transforming growth factor beta-3 (TGFβ3), and transmembrane protein 43 (TMEM43) Minor History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria ARVC confirmed pathologically or by current Task Force Criteria in second-degree relative Premature sudden death (<35 years of age) due to suspected ARVC in a first-degree relative Definite ARVC diagnosis requires: 2 major, 1 major + 2 minor or 4 minor criteria from different categories of the abovementioned criteria, in the absence of another cause of disease. Abbreviations: ARVC = arrhythmogenic right ventricular cardiomyopathy, ECG = electrocardiography, PLAX/PSAX = parasternal long/short axis view, RVOT = RV outflow tract, BSA = body surface area, RBBB/LBBB = right/left bundle branch block. Echocardiographic deformation imaging Echocardiographic deformation imaging has been proposed as an additional method for detection of early disease manifestation in ARVC.19 This technique has developed over the past two decades as a sensitive method for quantitative assessment of both global and regional contractile function of myocardial tissue. Acoustic markers called “speckles” are tracked in a two-dimensional image plane to estimate relative changes in myocardial length through
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