Table 1.
ORIGINAL TASK FORCE CRITERIA | REVISED TASK FORCE CRITERIA | |
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I. Global and/or Regional Dysfunction and Structural Alterations | I. Global and/or Regional Dysfunction and Structural Alterations* | |
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Regional RV akinesia, dyskinesia or aneurysm. | ||
Severe dilatation and reduction of right ventricular ejection fraction with no (or only mild) LV impairment. | And one of the following (end diastole): | |
Parasternal long axis view RVOT (PLAX) | ≥ 32 mm | |
Corrected for body size (PLAX/BSA) | ≥ 19 mm/m2 | |
Localized right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulging). | Parasternal short axis view RVOT (PSAX) | ≥ 36 mm |
Corrected for body size(PSAX/BSA) | ≥ 21 mm/m2 | |
or | ||
Severe segmental dilatation of the right ventricle. | ||
Fractional area change (FAC) | ≤ 33% | |
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Regional RV akinesia or dyskinesia or dyssynchronous RV contraction | ||
And one of the following: | ||
Right ventricular end diastolic volume (RVEDV/BSA) | ≥110 ml/m2 male | |
≥100 ml/m2 female | ||
OR | ||
Right ventricular ejection fraction (RVEF) | ≤40% | |
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Regional RV akinesia, dyskinesia or aneurysm | ||
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Regional RV akinesia or dyskinesia | ||
Mild global right ventricular dilatation and/or ejection fraction reduction with normal left ventricle. | And one of the following (end diastole): | |
Parasternal long axis view RVOT (PLAX) | ≥ 29 - < 32 mm | |
Mild segmental dilatation of the right ventricle. | Corrected for body size (PLAX/BSA) | ≥ 16 - < 19 mm/m2i |
Regional right ventricular hypokinesia. | Parasternal short axis view RVOT (PSAX) | ≥ 32 - < 36 mm |
Corrected for body size (PSAX/BSA) | ≥ 18 - < 21 mm/m2 | |
or | ||
Fractional area change (FAC) | > 33% - ≤ 40% | |
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Regional RV akinesia or dyskinesia or dyssynchronous RV contraction | ||
And one of the following: | ||
Right ventricular end diastolic volume/BSA | ≥100 - < 110 ml/m2 male | |
≥90 - < 100 ml/m2 female | ||
OR | ||
Right ventricular ejection fraction (RVEF) | > 40% - ≤ 45% | |
II. Tissue Characterization of Wall | II. Tissue Characterization of Wall | |
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Fibrofatty replacement of myocardium on endomyocardial biopsy. | Residual myocytes <60% by morphometric analysis, (or < 50% if estimated), with fibrous replacement of the RV free wall myocardium in at least 1 sample, with or without fatty replacement of tissue on endomyocardial biopsy. | |
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Residual myocytes 60 – 75% by morphometric analysis, (or 50 to 65% if estimated), with fibrous replacement of the RV free wall myocardium in at least 1 sample, with or without fatty replacement of tissue on endomyocardial biopsy. | ||
III. Repolarization Abnormalities | III. Repolarization Abnormalities | |
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Inverted T waves in right precordial leads (V2 and V3) (people aged >12 years, in absence of right bundle branch block). | Inverted T waves in right precordial leads (V1, V2 and V3) or beyond in individuals > 14 years of age (in the absence of complete right bundle branch block QRS ≥ 120 msecs). | |
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Inverted T waves in leads V1 and V2 in individuals > 14 years of age (in the absence of complete right bundle branch block), 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 bundle branch block. | ||
IV. Depolarization/Conduction Abnormalities | IV. Depolarization/Conduction Abnormalities | |
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Epsilon waves or localized prolongation (>110 ms) of the QRS complex in right precordial leads (V1 – V3). | 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) | |
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Late potentials (signal-averaged ECG). | Late potentials by signal averaged ECG in at least one of three parameters in the absence of a QRS duration of ≥110 msecs on the standard ECG. | |
Filtered QRS duration (fQRS) | ≥114 msecs | |
Duration of terminal QRS < 40 μV (LAS) | ≥38 msecs | |
RMS voltage of terminal 40 msecs | ≥20 μV | |
Terminal activation duration of QRS ≥ 55ms 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 right bundle branch block. | ||
V. Arrhythmias | V. Arrhythmias | |
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Left bundle branch block type ventricular tachycardia (sustained and nonsustained) (ECG, Holter, exercise testing). | Non-sustained or sustained VT of left bundle branch morphology with superior axis (negative or indeterminate QRS in II, III, AVF and positive in AVL) | |
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Non sustained or sustained VT of right ventricular outflow configuration, LBBB morphology with inferior axis (positive QRS in II, III, AVF and negative in AVL) or of unknown axis. | ||
Frequent ventricular extrasystoles (>1000/24 hours) (Holter). | Greater than 500 ventricular extrasystoles/24 hours by Holter | |
VI. Family History | VI. Family History | |
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Familial disease confirmed at necropsy or surgery. | 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 mutation † categorized as associated or probably associated with ARVC/D in the patient under evaluation. | ||
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Family history of premature sudden death (<35 years) due to suspected right ventricular dysplasia. | History of ARVC/D in a first degree relative in whom it is not possible or practical to determine if the family member meets current task force criteria. | |
Familial history (clinical diagnosis based on present criteria). | Premature sudden death (<35 years) due to suspected ARVC/D in a first degree relative. | |
ARVC/D confirmed pathologically or by current Task Force Criteria in second degree relative. |
Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria.
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.
Diagnostic terminology for original criteria | Diagnostic terminology for revised criteria |
This diagnosis is fulfilled by the presence of two major, or one major plus two minor criteria or four minor criteria from different groups |
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