Arrhythmogenic right ventricular dysplasia diagnostic criteria

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

There is no pathognomonic feature of ARVD. The use of MRI has improved in the diagnostic capabilities by virtue of its ability to characterize the RV free wall and the anatomy of the right ventricle. To establish the diagnosis of ARVD, it requires either 2 major criteria or 1 major and 2 minor criteria or 4 minor criteria. In 2010, a revision of the original Task Force Criteria incorporating imaging modalities and genetic testing was developed to improve diagnostic sensitivity while maintaining specificity.

Revised Task Force Diagnostic Criteria (2010)

The 1994 Task Force Criteria have been modified to incorporate imaging modalities and genetic testing to improve diagnostic sensitivity while maintaining specificity. In the revised criteria, quantitative metrics were implemented and abnormalities were defined on the basis of comparison with normal subject data. Scoring by major and minor criteria was maintained, structural abnormalities were quantified, and Task Force Criteria highly specific for ARVD were upgraded to major. Moreover, a number of criteria were added: terminal activation duration of QRS equal to or greater than 55 milliseconds, ventricular tachycardia (VT) with left bundle branch block (LBBB) morphology and superior axis, and genetic criteria.

Original and Revised Task Force Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Original Task Force Criteria (1994)		Revised Task Force Criteria (2010)
I. Global or Regional dysfunction and Structural Alterations*
Major	Severe dilatation and reduction of RV ejection fraction with no (or only mild) LV impairment Localized RV aneurysms (akinetic or dyskinetic areas with diastolic bulging) Severe segmental dilatation of the RV	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 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) — or RV ejection fraction ≤40% By RV angiography: Regional RV akinesia, dyskinesia, or aneurysm
Minor	Mild global RV dilatation and/or ejection fraction reduction with normal LV Mild segmental dilatation of the RV Regional RV hypokinesia	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) — or 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	Fibrofatty replacement of myocardium on endomyocardial biopsy	Residual myocytes <60% by morphometric analysis (or <50% if estimated), with fibrous replacement of the RV free wall myocardium in at least one 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 at least one 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
Minor	Inverted T waves in right precordial leads (V2 and V3) (people age >12 years, in absence of right bundle-branch block)	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 waves or localized prolongation (>110 ms) of the QRS complex in right precordial leads (V1 to V3)	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 (SAECG)	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 mV (low-amplitude signal duration) ≥38 ms Root-mean-square voltage of terminal 40 ms ≥20 mV 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 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	Left bundle-branch block-type ventricular tachycardia (sustained and nonsustained) (ECG, Holter, exercise) Frequent ventricular extrasystoles (>1000 per 24 hours) (Holter)	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	Familial disease confirmed at necropsy or surgery	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	Family history of premature sudden death (<35 years of age) due to suspected ARVC/D Familial history (clinical diagnosis based on present criteria)	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
Diagnostic terminology for original criteria: This diagnosis is fulfilled by the presence of 2 major, or 1 major plus 2 minor criteria or 4 minor criteria from different groups. Diagnostic terminology for revised criteria: — Definite diagnosis: 2 major or 1 major and 2 minor criteria or 4 minor from different categories — Borderline: 1 major and 1 minor or 3 minor criteria from different categories — Possible: 1 major or 2 minor criteria from different categories. *Hypokinesis is not included in this or subsequent definitions of RV regional wall motion abnormalities for the proposed modified criteria. †A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, is unobserved or rare in a large non-ARVC/D 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. PLAX, parasternal long-axis view; RVOT, RV outflow tract; BSA, body surface area; PSAX, parasternal short-axis view; aVF, augmented voltage unipolar left foot lead; and aVL, augmented voltage unipolar left arm lead.

Original Task Force Diagnostic Criteria (1994)

In 1994, the European Society of Cardiology and the International Society and Federation of Cardiology proposed diagnostic criteria for arrhythmogenic right ventricular dysplasia/cardiomyopathy. To make the diagnosis of ARVD/C, it requires either two major, one major and two minor, or four minor criteria.

Major Criteria

• Right ventricular dysfunction
  • Severe dilatation and reduction of RV ejection fraction with little or no LV impairment
  • Localized RV aneurysms
  • Severe segmental dilatation of the RV
• Tissue characterization
  • Fibrofatty replacement of myocardium on endomyocardial biopsy
• Conduction abnormalities
  • Epsilon waves in V₁ - V₃.
  • Localized prolongation (>110 ms) of QRS in V₁ - V₃
• Family history
  • Familial disease confirmed on autopsy or surgery

Minor Criteria

• Right ventricular dysfunction
  • Mild global RV dilatation and/or reduced ejection fraction with normal LV.
  • Mild segmental dilatation of the RV
  • Regional RV hypokinesis
• Tissue characterization
• Conduction abnormalities
  • Inverted T waves in V₂ and V₃ in an individual over 12 years old, in the absence of a right bundle branch block (RBBB)
  • Late potentials on signal averaged EKG
  • Ventricular tachycardia with a left bundle branch block (LBBB) morphology
  • Frequent PVCs (> 1000 PVCs / 24 hours)
• Family history
  • Family history of sudden cardiac death before age 35
  • Family history of ARVD

References

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