Evaluating the Revised 2010 Cardiac Magnetic Resonance Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy

Background: Making a clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is difficult and dependent on complex criteria. We previously showed that the revised cardiac magnetic resonance (CMR) criteria created a group of patients with isolated right ventricular (RV) dilation or dyskinesia that satisfied the original but not the revised criteria. Methods: We re-assessed 55 patients with clinical suspicion of ARVC and either isolated RV dilation (n = 23) or RV dyskinesia (n = 32). Mean follow up time was 69 ± 25 months. Serial CMR was performed on 50 patients; 5 were excluded due to an implanted cardiac defibrillator (ICD). Results: There was improvement in indexed RV end diastolic volume, indexed RV end systolic volume and RVEF irrespective of body surface area; −11.7±15.2 mls/m2, −6.4±10.5 mls/m2 and +3.3±7.9% (p values = 0.004, 0.017 and 0.031). Of note in follow up, 29 (59%) patients had normalised RV parameters, 19 (39%) maintained the same degree of abnormality and 2 (4%) progressed to satisfy the major ARVC criteria. The negative predictive value of the revised CMR criteria is 96%. All five patients excluded with ICD recorded no shocks and clinically did not evolve into ARVC. Conclusion: These data support the accuracy of the revised CMR criteria for ARVC and its potential use as a ‘rule out’ test owing to its high negative predictive value. Serial CMR imaging recommended for those with high-risk features but not diagnostic RV abnormalities for ARVC.