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New Diagnostic Criteria for Arrhythmogenic Cardiomyopathy

These new insights into the heterogeneous genetic mutations and phenotypic manifestations of ACM led to a critical revision of the 2010 ITF criteria, which exclusively targeted RV classical forms and did not include the tissue characterization by contrast enhanced cardiac magnetic resonance (CMR) imaging. Accordingly, an International Expert consensus document has been recently developed to provide upgraded criteria (“the Padua Criteria”) for the diagnosis of the whole spectrum of ACM phenotypes.

“Padua criteria” for diagnosis of arrhythmogenic cardiomyopathy

Category Right Ventricle Left Ventricle
I. Morpho-functional ventricular abnormalities By echocardiography, CMR or angiography:
Major
• Regional RV akinesia, dyskinesia, or bulging, plus, one of the following:
– global RV dilatation (increase of RV EDV according to the imaging test specific nomograms)
– global RV systolic dysfunction (reduction of RV ejection fraction (EF) according to the imaging test specific nomograms)
Minor
• Regional RV akinesia, dyskinesia, or aneurysm of RV free wall
By echocardiography, CMR or angiography:
Minor
• Global LV systolic dysfunction (depression of LV EF or reduction of echocardiographic global longitudinal strain), with or without LV dilatation (increase of LV EDV according to the imaging test specific nomograms for age, sex, and body surface area (BSA))
Minor
• Regional LV hypokinesia or akinesia of LV free wall, septum, or both
II. Structural myocardial abnormalities By CE-CMR:
Major
• Transmural LGE (stria pattern) of ≥1 RV region(s) (inlet, outlet, and apex in 2 orthogonal views)
By EMB (limited indications):
Major
• Fibrous replacement of the myocardium in ≥1 sample, with or without fatty tissue
By CE-CMR:
Major
• LV LGE (stria pattern) of ≥1 Bull’s Eye segment(s) (in 2 orthogonal views) of the free wall (subepicardial or midmyocardial), septum, or both (excluding septal junctional LGE
III. Repolarization abnormalities Major
• Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individuals with complete pubertal development (in the absence of complete RBBB)
Minor
• Inverted T waves in leads V1 and V2 in individuals with completed pubertal development (in the absence of complete RBBB)
• Inverted T waves in V1, V2, V3 and V4 in individuals with completed pubertal development in the presence of complete RBBB.
Minor
Inverted T waves in left precordial leads (V 4–V 6) (in the absence of complete LBBB)
IV. Depolarization abnormalities Minor
• 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)
• 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)
Minor
• Low QRS voltages (<0.5 mV peak to peak) in limb leads (in the absence of obesity, emphysema, or pericardial effusion)
V. Ventricular arrhythmias Major
• Frequent ventricular extrasystoles (>500 per 24 h), non-sustained or sustained ventricular tachycardia of LBBB morphology
Minor
• Frequent ventricular extrasystoles (>500 per 24 h), non-sustained or sustained ventricular tachycardia of LBBB morphology with inferior axis (“RVOT pattern”)
Minor
• Frequent ventricular extrasystoles (>500 per 24 h), non-sustained or sustained ventricular tachycardia with a RBBB morphology (excluding the “fascicular pattern”)
VI. Family history/genetics Major
• ACM confirmed in a first-degree relative who meets diagnostic criteria
• ACM confirmed pathologically at autopsy or surgery in a first degree relative
• Identification of a pathogenic or likely pathogenetic ACM mutation in the patient under evaluation
Minor
• History of ACM in a first-degree relative in whom it is not possible or practical to determine whether the family member meets diagnostic criteria
• Premature sudden death (<35 years of age) due to suspected ACM in a first-degree relative
• ACM confirmed pathologically or by diagnostic criteria in a second-degree relative

ACM = arrhythmogenic cardiomyopathy; BSA = body surface area; EDV = end diastolic volume; EF = ejection fraction; ITF = International Task Force; LBBB = left bundle-branch block; LGE = late gadolinium
enhancement; LV = left ventricle; RBBB = right bundle-branch block; RV = right ventricle; RVOT = right ventricular outflow tract; CE-CMR = contrast enhanced-cardiovascular magnetic resonance; EMB = endomyocardial biopsy

 

References:

  1. Mattesi G, Cipriani A, Bauce B, Rigato I, Zorzi A, Corrado D. Arrhythmogenic Left Ventricular Cardiomyopathy: Genotype-Phenotype Correlations and New Diagnostic Criteria. J Clin Med. 2021 May 20;10(10):2212. [Medline]
  2. Corrado D, Zorzi A, Cipriani A, Bauce B, Bariani R, Beffagna G, De Lazzari M, Migliore F, Pilichou K, Rampazzo A, Rigato I, Rizzo S, Thiene G, Perazzolo Marra M, Basso C. Evolving Diagnostic Criteria for Arrhythmogenic Cardiomyopathy. J Am Heart Assoc. 2021 Sep 21;10(18):e021987. [Medline]

 

Created Nov 26, 2021.

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