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By 2D echo: • Regional RV akinesia, dyskinesia, or aneurysm • and 1 of the following (end diastole): - PLAX RVOT ≥32 mm (corrected for body size [PLAX/BSA] ≥19 mm/m 2 ) - PSAX RVOT ≥36 mm (corrected for body size [PSAX/BSA] ≥21 mm/m 2 ) - or fractional area change ≤33 percent

By MRI: • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction • and 1 of the following: - Ratio of RV end-diastolic volume to BSA ≥110 mL/m 2 (male) or ≥100 mL/m 2 (female) - or RV ejection fraction ≤40 percent

By 2D echo: • Regional RV akinesia or dyskinesia • and 1 of the following (end diastole): - PLAX RVOT ≥29 to <32 mm (corrected for body size [PLAX/BSA] ≥16 to <19 mm/m 2 ) - PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m 2 ) - or fractional area change >33 percent to ≤40 percent

By MRI: • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction • and 1 of the following: - Ratio of RV end-diastolic volume to BSA ≥100 to <110 mL/m 2 (male) or ≥90 to <100 mL/m 2 (female) - or RV ejection fraction >40 percent to ≤45 percent II. Tissue characterization of wall

Major • Residual myocytes <60 percent by morphometric analysis (or <50 percent if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy

Minor • Residual myocytes 60 percent to 75 percent by morphometric analysis (or 50 percent to 65 percent if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy III. Repolarization abnormalities

Minor • Inverted T waves in leads V 1 and V 2 in individuals >14 years of age (in the absence of complete right bundle-branch block) or in V 4 , V 5 , or V 6 • Inverted T waves in leads V 1 , V 2 , V 3 , and V 4 in individuals >14 years of age in the presence of complete right bundle-branch block IV. Depolarization/conduction abnormalities

Minor • Late potentials by SAECG in ≥1 of the following 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 V 1 , V 2 , or V 3 , in the absence of complete right bundle-branch block V. Arrhythmias

Minor • Nonsustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis • >500 ventricular extrasystoles per 24 hours (Holter) VI. Family history

Major • ARVC/D confirmed in a first-degree relative who meets current Task Force criteria • ARVC/D confirmed pathologically at autopsy or surgery in a first-degree relative • Identification of a pathogenic mutationΔ categorized as associated or probably associated with ARVC/D in the patient under evaluation

Minor • History of ARVC/D in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria • Premature sudden death (<35 years of age) due to suspected ARVC/D in a first-degree relative • ARVC/D confirmed pathologically or by current Task Force Criteria in second-degree relative Diagnostic terminology for revised criteria: Definite diagnosis: 2 Major, OR 1 Major and 2 Minor criteria, OR 4 Minor from different categories Borderline diagnosis: 1 Major and 1 Minor, OR 3 Minor criteria from different categories Possible diagnosis: 1 Major, OR 2 Minor criteria from different categories PLAX: parasternal long-axis view; RVOT: RV outflow tract; BSA: body surface area; PSAX: parasternal short-axis view; aVF: augmented voltage unipolar left foot lead; aVL: augmented voltage unipolar left arm lead. * Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria. Δ A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, is unobserved or rare in a large non-ARVC/D control population, and either alters or is predicted to alter the structure or function of the protein or has demonstrated linkage to the disease phenotype in a conclusive pedigree. Modified with permission from: Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force criteria. Circulation 2010; 121:1533. Copyright © 2010 Lippincott Williams & Wilkins.