There is no pathognomonic feature of ARVC. The diagnosis of ARVC is based on a combination of major and minor criteria.
To make a diagnosis of ARVC requires either 2 major criteria or 1 major and 2 minor criteria or 4 minor criteria.
The criteria were updated in 2010. This is readily calculated at the offical
online calculator: TFC-calculator.ca
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) fractional area change ≤33% By MRI: 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) 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 mm/m²) PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m²) fractional area change >33% to ≤40% By MRI: 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 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
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 right bundle-branch block QRS ≥120 ms) 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 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 Terminal activation duration of QRS ≥55 ms measured from the nadir of the S wave to the end of the QRS including RŽ, in V1, V2, or V3, in the absence of complete right bundle-branch block
V. 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) VI. Family history MAJOR ♥ 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 MINOR ♥ 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 (<35 years of age) due to suspected ARVC/D in a first-degree relative ARVC/D confirmed pathologically or by current Task Force Criteria in second-degree relative
