The following are key points to remember from this current opinion article on evaluation of the current diagnostic criteria and differential diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC):

1. ARVC, previously considered to be a primary right ventricular (RV) disease, also has biventricular and primarily left ventricular (LV) subtypes.
2. The 2010 International Task Force Diagnostic Criteria for ARVC contain several limitations and do not fully consider these non-RV subtypes. This review critically appraises the criteria and provides recommendations to improve evaluation of ARVC.
3. The pathogenicity of the various gene mutations associated with ARVC is not completely defined. Caution should be exercised in classifying a gene mutation as a pathogenic in patients without other phenotypic findings of ARVC.
4. Endomyocardial biopsy and endocardial voltage mapping are invasive and difficult to interpret; these should be reserved for selected patients in which diagnosis is elusive.
5. Cardiac magnetic resonance imaging should include tissue characterization, which may be definitively indicative of fibro-fatty replacement in the right or left ventricles.
6. The interpretation of surveillance monitoring for ventricular arrhythmias should consider the possibility of either RV or LV origins in the various disease subtypes.
7. Electrocardiogram (ECG)-specific findings may suggest RV versus LV disease. Low QRS voltage in the limb leads is a marker of LV involvement.
8. The diagnosis of ARVC in pediatric patients is difficult due to the low prevalence of phenotypic disease. The evaluation of child relatives of probands should include genetic evaluation and routine surveillance during childhood to evaluate for developing disease.
9. RV outflow tract ventricular tachycardia and Brugada syndrome may present similar arrhythmias as ARVC and should be considered in the differential diagnosis.
10. RV volume overload due to congenital heart lesions may result in findings similar to ARVC.
11. Various structural diseases such as myocarditis and sarcoidosis may mimic the findings of LV predominant ARVC.
12. The more global designation of “arrhythmogenic cardiomyopathy” should indicate the family of genetic diseases that include the subtypes of ARVC, LV arrhythmogenic cardiomyopathy, and biventricular cardiomyopathy.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, Acute Heart Failure, Magnetic Resonance Imaging

Keywords: Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Brugada Syndrome, Cardiomyopathies, Diagnosis, Differential, Electrocardiography, Genetic Testing, Heart Defects, Congenital, Heart Failure, Magnetic Resonance Imaging, Mutation, Myocarditis, Sarcoidosis, Pediatrics, Tachycardia, Ventricular

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