Impact of the revision of arrhythmogenic right ventricular cardiomyopathy/dysplasia task force criteria on its prevalence by CMR criteria

Abstract

Objectives: The purpose of our study was to assess the impact of revised versus original criteria on the prevalence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) criteria in cardiac magnetic resonance (CMR) studies.

Background: Recently, the ARVC/D task force criteria have been revised, aiming for a better diagnostic sensitivity. The implications of this revision on clinical decision making are unknown.

Methods: We retrospectively evaluated the CMR scans of 294 patients referred for ARVC/D between 2005 and 2010, and determined the presence or absence of major and minor CMR criteria using the original and the revised task force criteria. Previously, major and minor abnormalities were identified by the presence of right ventricle dilation (global or segmental), right ventricle microaneurysm, or regional hypokinesis. The revised criteria require the combination of severe regional wall motion abnormalities (akinesis or dyskinesis or dyssynchrony) with global right ventricle dilation or dysfunction (quantitative assessment).

Results: Applying the original criteria, 69 patients (23.5%) had major original criteria, versus 19 patients (6.5%) with the revised criteria. Forty-three patients (62.3%) with major original criteria did not meet any of the revised criteria. Using the original criteria, 172 patients (58.5%) had at least 1 minor criterion versus 12 patients (4%) with the revised task force criteria; 167 patients (97%) with minor original criteria did not meet any of the revised criteria. In the subgroup of 134 patients with complete diagnostic work-up of ARVC, 10 patients met the diagnosis of proven ARVC/D without counting imaging criteria. Only 4 of 10 met major criteria according to the revised CMR criteria; none met minor criteria. However, 112 of 124 patients without ARVC/D were correctly classified as negative by major and minor criteria (specificity 94% and 96%, respectively).

Conclusions: In our experience, the revision of the ARVC/D task force imaging criteria significantly reduced the overall prevalence of major and minor criteria. The revision, although maintaining a high specificity, may not have improved the sensitivity for identifying patients with ARVC/D. Larger studies including follow-up are required.