Marcus, FI. et al. Circulation 2010;121:1533-41. (Revised criteria - listed below (Article is available for free)
Diagnostic levels: (Definite, Borderline and Possible)
Definite diagnosis - 2 majors or 1 major & 2 minor criteria or 4 minor criteria from diff. categories.
Borderline diagnosis - 1 major & 1 minor, or 3 minor criteria from different categories
Possible diagnosis - 1 major or 2 minor criteria from different categories.
The diagnostic criteria fall under 6 different categories:
I) Dysfunction or Structural alterations
II) Tissue characterization
III) Repolarization abnormalities
IV) Depolarization abnormalities
V) Arrhythmias
VI) Family history
I) Dysfunction or Structural alterations
Major -By 2D echo:
(i) Regional RV akinesia, dyskinesia or aneurysm & one of the following three
(a) PLAx (end diastole) RVOT ≥ 32 mm (≥19 mm/m2)
(b) PSAx (end diastole) RVOT ≥ 36 mm (≥ 21 mm/m2)
(c) ≤ FAC 33%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV ≥ 110 ml/m2 for male, ≥ 100 ml/m2 for female
(b) ≤ RVEF 40%
By RV angiography:
(i) Regional RV akinesia, dyskinesia or aneurysm.
Minor -
By 2D echo:
(i) Regional RV akinesia or dyskinesia & one of the following three
(a) PLAx (end diastole) RVOT 29 - 32 mm (16 - 19 mm/m2)
(b) PSAx (edn diastole) RVOT 32 - 36 mm (18 - 21 mm/m2)
(c) ≤ FAC 33 - 40%
By MRI:
(i) Regional RV akinesia or dyskinesia or dyssynchronious RV contraction & one of the following two.
(a) RVEDV 100 - 110 ml/m2 for male, 90 - 100 ml/m2 for female
(b) RVEF 40 - 45%
II) Tissue characterization (Fibrofatty replacement of myocardium)
Major -(i) Biopsy - Residual myocytes < 60% by morphometric analysis (or < 50% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement)
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without="">Minor -
(i) Biospy - Residual myocytes 60-75% by morphometric analysis or 50-65% if estimated) with fibrous replacement of RV free wall myocardium (with or without fatty replacement).
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> III) Repolarization abnormalities:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) T wave inversion in V1-V3 or beyond (in >14 yrs of age, in the absence of RBBB and QRS ≥120 ms).Minor -
(i) T wave inversion in V1-V2 (in >14 yrs of age, absence of RBBB) or in V4, V5 or V6.
(ii) T wave inversion in V1 - V4 with RBBB.
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> IV) Depolarization abnormalities:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major -(i) Epsilon wave in V1 - V3 (Reproducible, low-amplitude signals between QRS and T wave)
Minor -
(i) Late potential in Signal-averaged ECG (in ≥ 1 of 3 parameters in the absence of a QRS ≥ 110 ms in standard ECG)
(ii) Filtered QRS duration (fQRS) ≥ 114 ms.
(iii) Duration of terminal QRS (< 40 µV) ≥ 38 ms.
(iv) Root-Mean-Square voltage of terminal 40 ms ≤ 20 µV
(v) Terminal activation duration of QRS ≥ 55 ms measured from nadir of the S wave to the end of the QRS, including R' in V1, V2 or V3 in the absence of RBBB.
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> V) Arrhythmias:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) V-Tach with LBBB morphology with superior axis (negative in II, III and aVF & positive in aVL)Minor -
(i) V-Tach with RVOT morphology i.e. LBBB morphology with inferior axis (positive in II, III and aVF & negative in aVL) or unknown axis.
(ii) > 500 PVCs in 24 hrs. (Holter)
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> VI) Family history:
<50 estimated="" fatty="" if="" myocardium.="" of="" or="" p="" replacement="" with="" without=""> Major - (i) First-degree relative with ARVC/D confirmed by current criteria(ii) First-degree relative with ARVC/D confirmed by surgery or autopsy
(iii) Pathogenic mutation (associated or probably associated with ARVC/D) found in the patient under evaluation (Technically, this is not "family history")
Minor -
(i) First-degree relative with history of ARVC/D that cannot be confirmed with current criteria
(ii) First-degree relative with sudden death < 35 years of age in whom ARVC/D was suspected as cause
(iii) Second-degree relative with confirmed ARVC/D with current criteria or by pathology
✪Treatment of ARVC/D - International Consensus Statement. Circulation 2015;132:441-53. (Article available for free).
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