Protocol
  1. Anatomy module (A)
  2. LV function module (B)
  3. RV function module (C), axial and RVOT
    • Slice thickness 6-8 mm without inter-slice gap
  4. T1w axial black blood images (optional)
  5. T1w axial fat suppressed black blood images (optional)
  6. LGE module (E) in same orientations
    • T1 nulling for RV
Report
  1. Dimensions, mass (corrected for BSA) and function
    • LV: EDV, ESV , SV, EF, longitudinal function, mass
    • RV: EDV, ESV, SV, EF, longitudinal function
    • RV regional wall motion abnormalities (inflow, apex, outflow)
  2. Presence of morphological RV abnormalities (aneurysms, outpouchings)
  3. Presence of fatty RV or LV infiltration (if acquired)
  4. Presence and extent of fibrosis
Key Points
  1. Diagnosis cannot be based on imaging criteria alone
    - See modified Task Force ARVC criteria
  2. RV wall motion abnormalities at the moderator band insertion point is common in normal subjects
Diagnostic Criteria
    Definite diagnosis
  • 2 major or 1 major and 2 minor criteria
  • 4 minor criteria
    Borderline diagnosis
  • 1 major and 1 minor
  • 3 minor criteria
    Possible diagnosis
  • 1 major or 2 minor criteria

1. Global or regional dysfunction and structural alterations
    Major
  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • ...and 1 of the following:
    • Ratio of RV EDV to BSA ≥110mL/ m2(male) or ≥100mL/m2(female)
    • or RV ejection fraction ≤40%
    Minor
  • Regional RV akinesia or dyskinesia or dyssynchronous RV contraction
  • ...and 1 of the following:
    • Ratio of RV EDV to BSA ≥100 to <110mL/m2 (male) or ≥90 to <100mL/m2 (female)
    • or RV EF >40% to ≤45%
2. Tissue characterization of wall (histological)
    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 endomyocardial biopsy
    Minor
  • Residual myocytes 60% to 75% by morphometric analysis (or 50% to 65% if estimated), with fibrous replacement of the RV free wall myocardium in ≥1 sample, with or without fatty replacement of tissue on endomyocardial biopsy
3. 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 right bundle-branch block QRS ≥120ms)
    Minor
  • 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
4. 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 ≥110ms on the standard ECG
  • Filtered QRS duration (fQRS) ≥114ms
  • Duration of terminal QRS <40μV (low-amplitude signal duration) ≥38ms
  • Root-mean-square voltage of terminal 40ms ≤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
5. Arrhythmias
    Major
  • 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)
    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)
6. Family history
    Major
  • ARVC confirmed in a first-degree relative who meets current Task Force criteria
  • ARVC confirmed pathologically at autopsy or surgery in a first- degree relative
  • Identification of a pathogenic mutation* categorized as associated or probably associated with ARVC in the patient under evaluation
    Minor
  • History of ARVC 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 in a first-degree relative
  • ARVC confirmed pathologically or by current Task Force Criteria in second-degree relative
Tips and Tricks
  1. Focus on RV volumes and functional RV abnormalities
  2. Consider antiarrhythmic drugs in patients with VES
  3. Consider alternative causes (abnormal vascular connections / shunts) in patients with dilated RV