Impact of the Revision of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Task Force Criteria on Its Prevalence by CMR Criteria

Study Questions:

How have the revised task force criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) influenced the sensitivity and specificity of cardiac magnetic resonance (CMR)?


This was a retrospective analysis of 294 patients (mean age 43 years) who underwent CMR for evaluation of ARVC/D. CMRs were reviewed using the original and revised TFC.


Major criteria for ARVC/D were present in 23.5% of patients using the original TFC compared to 6.5% using the revised criteria. This was due mostly to exclusion of regional wall motion abnormalities and microaneurysms in the revised TFC. Minor CMR criteria were present in 58.5% of patients using the original TFC compared to 4% with the revised criteria. Among 10 patients who met clinical criteria for ARVC/D, major CMR criteria were present in 90% of patients using the original TFC compared to 40% using the revised TFC.


The revised TFC for ARVC/D increase specificity, but decrease the sensitivity of CMR for the detection of ARVC/D.


The original TFC included severe right ventricular (RV) dilatation and reduced RV ejection fraction (EF), or localized RV aneurysms, or severe segmental RV dilatation. The revised TFC are stricter, in that regional RV akinesia or dyskinesia must be accompanied by RV volumes greater than specific cut-off values or an RVEF ≤40%. The revised criteria are less subjective, leading to greater specificity for the diagnosis of ARVC/D. This study suggests that sensitivity is significantly compromised with the revised CMR TFC. This could be a clinical problem if the diagnosis of ARVC/D depended only on CMR, which is not the case.

Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias

Keywords: Arrhythmogenic Right Ventricular Dysplasia, Prevalence, Ventricular Function, Right, Sensitivity and Specificity, Dyskinesias, Magnetic Resonance Spectroscopy

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