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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

 

▪ 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

 

▪ 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

 

▪ 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

 

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

 

▪ 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

 

▪ 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

 

▪ 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