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Table 1 2010 Revised Task Force Criteria for AC

From: Arrhythmogenic cardiomyopathy

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)

 

▪ or fractional area change ≤ 33 %

 

By CMR

 

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)

 

▪ or 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 m/m2)

 

▪ PSAX RVOT ≥32 to <36 mm (corrected for body size [PSAX/BSA] ≥18 to <21 mm/m2)

 

▪ or fractional area change >33 % to ≤40 %

 

By CMR

 

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 EMB

Minor

 

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 EMB

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 RBBB QRS ≥120 ms)

Minor

 

▪ Inverted T waves in leads V1 and V2 in individuals >14 years of age (in the absence of complete RBBB) 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 RBBB

IV. Depolarization/conduction abnormalities

Major

 

â–ª 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 of QRS ≥55 ms 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 RBBB

V. Arrhythmias

Major

 

â–ª Nonsustained or sustained VT of LBBB morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL)

Minor

 

â–ª Nonsustained or sustained VT of RVOT configuration, LBBB morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis

 

▪ >500 PVCs per 24 hours (Holter)

VI. Family history

Major

 

â–ª AC confirmed in a first-degree relative who meets current Task Force criteria

 

â–ª AC confirmed pathologically at autopsy or surgery in a first-degree relative

 

▪ Identification of a pathogenic mutation† categorized as associated or probably associated with AC in the patient under evaluation

Minor

 

â–ª History of AC 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 SD (35 years of age) due to suspected AC in a first-degree relative

 

â–ª AC confirmed pathologically or by current Task Force Criteria in second-degree relative

  1. Two major, or one major and two minor, or four minor criteria: definite diagnosis of AC. One major and one minor, or three minor criteria: borderline diagnosis; One major, or two minor criteria from different categories: possible diagnosis
  2. * Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria
  3. † A pathogenic mutation is a DNA alteration associated with AC that alters or is expected to alter the encoded protein, is unobserved or rare in a large non-AC 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
  4. Abbreviations. BSA: body surface area; CMR: cardiac magnetic resonance; EMB: endomyocardial biopsy; LBBB: left bundle- branch block; PLAX: parasternal long-axis view; PSAX: parasternal short-axis view; PVC: premature ventricular complex; RBBB: right bundle-branch block; RV: right ventricle; RVOT: RV outflow tract; SD: sudden death; VT: ventricular tachycardia