Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions

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{| class="wikitable" style="width:700px"
{| class="wikitable" style="width:700px"
|+ Comparison of Original and Revised Task Force Criteria
|+ Comparison of Original and Revised Task Force Criteria
! style="width:500px" | Original Task Force Criteria
! colspan="2" style="width:600px" | Revised Task Force Criteria
! style="width:500px" | Revised Task Force Criteria
|-
|-
| colspan="2" | '''I. Global or regional dysfunction and structural alterations<sup>&lowast;</sup>'''
| colspan="2" | '''I. Global or regional dysfunction and structural alterations<sup>&lowast;</sup>'''
|-
|-
| colspan="2" style="padding-left:12px" | '''Major'''
| style="width:300px" | '''Major'''
| style="width:300px" |'"Minor'"
|-
|-
| valign="top" style="padding-left:24px" |
| valign="top" style="padding-left:24px" |
* Severe dilatation and reduction of RV ejection fraction with no (or only mild) LV impairment
* Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulging)
* Severe segmental dilatation of the RV
| valign="top" |
<strong>By 2D echo:</strong>
<strong>By 2D echo:</strong>
* Regional RV akinesia, dyskinesia, or aneurysm
* Regional RV akinesia, dyskinesia, or aneurysm
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<strong>By RV angiography:</strong>
<strong>By RV angiography:</strong>
* Regional RV akinesia, dyskinesia, or aneurysm
* Regional RV akinesia, dyskinesia, or aneurysm
|-
| valign="top" |  
| colspan="2" style="padding-left:12px"  | Minor
|-
| valign="top" style="padding-left:24px"  |
* Mild global RV dilatation and/or ejection fraction reduction with normal LV
* Mild segmental dilatation of the RV
* Regional RV hypokinesia
| valign="top" |
<strong>By 2D echo:</strong>
<strong>By 2D echo:</strong>
* Regional RV akinesia or dyskinesia
* Regional RV akinesia or dyskinesia
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| colspan="2" | '''II. Tissue characterization of wall'''
| colspan="2" | '''II. Tissue characterization of wall'''
|-
|-
| colspan="2" style="padding-left:12px" | '''Major'''
| '''Major'''
| '''Minor'''  
|-
|-
| valign="top" style="padding-left:24px" |
| valign="top" |
* Fibrofatty replacement of myocardium on endomyocardial biopsy
|
* Residual myocytes &lt;60% by morphometric analysis (or &lt;50% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
* Residual myocytes &lt;60% by morphometric analysis (or &lt;50% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
|-
| valign="top" |  
| colspan="2" style="padding-left:12px" | Minor
|-
|
| valign="top" |
* Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
* Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in &ge;1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
|-
|-
| colspan="2" | '''III. Repolarization abnormalities'''
| colspan="2" | '''III. Repolarization abnormalities'''
|-
|-
|'''Bold text''' colspan="2" style="padding-left:12px" | '''Major'''
| '''Major'''  
| '''Minor'''  
|-
|-
| &nbsp;
| valign="top" |
|
* Inverted T waves in right precordial leads (V<sub>1</sub>, V<sub>2</sub>, and V<sub>3</sub>) or beyond in individuals &gt;14 years of age (in the absence of complete right bundle-branch block QRS &ge;120 ms)
* Inverted T waves in right precordial leads (V<sub>1</sub>, V<sub>2</sub>, and V<sub>3</sub>) or beyond in individuals &gt;14 years of age (in the absence of complete right bundle-branch block QRS &ge;120 ms)
|-
| valign="top" |  
| colspan="2" style="padding-left:12px" | Minor
|-
| valign="top" style="padding-left:24px" |
* Inverted T waves in right precordial leads (V<sub>2</sub> and V<sub>3</sub>) (people age &gt;12 years, in absence of right bundle-branch block)
|
* Inverted T waves in leads V<sub>1</sub> and V<sub>2</sub> in individuals &gt;14 years of age (in the absence of complete right bundle-branch block) or in V<sub>4</sub>, V<sub>5</sub>, or V<sub>6</sub>
* Inverted T waves in leads V<sub>1</sub> and V<sub>2</sub> in individuals &gt;14 years of age (in the absence of complete right bundle-branch block) or in V<sub>4</sub>, V<sub>5</sub>, or V<sub>6</sub>
* Inverted T waves in leads V<sub>1</sub>, V<sub>2</sub>, V<sub>3</sub>, and V<sub>4</sub> in individuals &gt;14 years of age in the presence of complete right bundle-branch block
* Inverted T waves in leads V<sub>1</sub>, V<sub>2</sub>, V<sub>3</sub>, and V<sub>4</sub> in individuals &gt;14 years of age in the presence of complete right bundle-branch block
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| colspan="2" | '''IV. Depolarization/conduction abnormalities'''
| colspan="2" | '''IV. Depolarization/conduction abnormalities'''
|-
|-
| colspan="2" style="padding-left:12px" | '''Major'''
| '''Major'''
| '''Minor'''  
|-
|-
| style="padding-left:24px" |
| valign="top" |
* Epsilon waves or localized prolongation (&gt;110 ms) of the QRS complex in right precordial leads (V<sub>1</sub> to V<sub>3</sub>)
|
* Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V<sub>1</sub> to V<sub>3</sub>)
* Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V<sub>1</sub> to V<sub>3</sub>)
|-
| valign="top |
| colspan="2" style="padding-left:12px" | Minor
|-
| style="padding-left:24px" valign="top" |
* Late potentials (SAECG)
|
* Late potentials by SAECG in &ge;1 of 3 parameters in the absence of a QRS duration of &ge;110 ms on the standard ECG
* Late potentials by SAECG in &ge;1 of 3 parameters in the absence of a QRS duration of &ge;110 ms on the standard ECG
* Filtered QRS duration (fQRS) &ge;114 ms
* Filtered QRS duration (fQRS) &ge;114 ms
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| colspan="2" | '''V. Arrhythmias'''
| colspan="2" | '''V. Arrhythmias'''
|-
|-
| colspan="2" style="padding-left:12px" | '''Major'''
| '''Major'''
| '''Minor'''  
|-
|-
|
| valign="top" |
|
* Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL)
* Nonsustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL)
|-
| valign="top |
| colspan="2" style="padding-left:12px" | Minor
|-
| style="padding-left:24px" valign="top" |
* Left bundle-branch block–type ventricular tachycardia (sustained and nonsustained) (ECG, Holter, exercise)
* Frequent ventricular extrasystoles (&gt;1000 per 24 hours) (Holter)
|
* 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
* 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
* &gt;500 ventricular extrasystoles per 24 hours (Holter)
* &gt;500 ventricular extrasystoles per 24 hours (Holter)
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| colspan="2" | '''VI. Family history'''
| colspan="2" | '''VI. Family history'''
|-
|-
| colspan="2" style="padding-left:12px" | '''Major'''
| '''Major'''
| '''Minor'''  
|-
|-
| style="padding-left:24px" valign="top" |
| valign="top" |
* Familial disease confirmed at necropsy or surgery
|
* ARVC/D confirmed in a first-degree relative who meets current Task Force criteria
* 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
* ARVC/D confirmed pathologically at autopsy or surgery in a first-degree relative
* Identification of a pathogenic mutation<sup>&dagger;</sup> categorized as associated or probably associated with ARVC/D in the patient under evaluation
* Identification of a pathogenic mutation<sup>&dagger;</sup> categorized as associated or probably associated with ARVC/D in the patient under evaluation
|-
| valign="top |
| colspan="2" style="padding-left:12px" | Minor
|-
| style="padding-left:24px" valign="top" |
* Family history of premature sudden death (&lt;35 years of age) due to suspected ARVC/D
* Familial history (clinical diagnosis based on present criteria)
|
* 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
* 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 (&lt;35 years of age) due to suspected ARVC/D in a first-degree relative
* Premature sudden death (&lt;35 years of age) due to suspected ARVC/D in a first-degree relative