I. Global or regional dysfunction and structural alterations* | |
Major | |
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/m2) | |
▪ PSAX RVOT ≥36 mm (corrected for body size [PSAX/BSA] ≥21 mm/m2) | |
▪ or fractional area change ≤ 33 % | |
By CMR | |
Regional RV akinesia or dyskinesia or dyssynchronous RV contraction | |
and 1 of the following: | |
▪ Ratio of RV end-diastolic volume to BSA ≥110 mL/m2 (male) or ≥100 mL/m2 (female) | |
▪ or RV ejection fraction ≤40 % | |
By RV angiography | |
Regional RV akinesia, dyskinesia, or aneurysm | |
Minor | |
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 m/m2) | |
▪ PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m2) | |
▪ or fractional area change >33 % to ≤40 % | |
By CMR | |
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/m2 (male) or ≥90 to <100 mL/m2 (female) | |
▪ or RV ejection fraction >40 % to ≤45 % | |
II. Tissue characterization of wall | |
Major | |
Residual myocytes <60 % by morphometric analysis (or <50 % if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on EMB | |
Minor | |
Residual myocytes <60 % by morphometric analysis (or <50 % if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on EMB | |
III. Repolarization abnormalities | |
Major | |
▪ Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individuals >14 years of age (in the absence of complete RBBB QRS ≥120 ms) | |
Minor | |
▪ Inverted T waves in leads V1 and V2 in individuals >14 years of age (in the absence of complete RBBB) or in V4, V5, or V6 | |
▪ Inverted T waves in leads V1, V2, V3, and V4 in individuals >14 years of age in the presence of complete right RBBB | |
IV. Depolarization/conduction abnormalities | |
Major | |
▪ Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 to V3) | |
Minor | |
▪ Late potentials by SAECG 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 | |
V. Arrhythmias | |
Major | |
▪ Nonsustained or sustained VT 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 VT of RVOT configuration, LBBB morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis | |
▪ >500 PVCs per 24 hours (Holter) | |
VI. Family history | |
Major | |
▪ AC confirmed in a first-degree relative who meets current Task Force criteria | |
▪ AC confirmed pathologically at autopsy or surgery in a first-degree relative | |
▪ Identification of a pathogenic mutation† categorized as associated or probably associated with AC in the patient under evaluation | |
Minor | |
▪ History of AC 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 SD (35 years of age) due to suspected AC in a first-degree relative | |
▪ AC confirmed pathologically or by current Task Force Criteria in second-degree relative |