Is cardiac MRI an effective test for arrhythmogenic right ventricular cardiomyopathy diagnosis?

To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) and compare cardiac MR (cMR) findings against clinical diagnosis.

A retrospective analysis of 114 (age range 16 to 83, males 55% and females 45%) patients referred for cMR with a suspected diagnosis of ARVC between May 2006 and February 2010 was performed after obtaining institutional approval for service evaluation. Reasons for referral including clinical symptoms and family history of sudden death, electrocardiogram and echo abnormalities, cMR findings, final clinical diagnosis and information about clinical management were obtained. The results of cMR were classified as major, minor, non-specific or negative depending on both functional and tissue characterisation and the cMR results were compared against the final clinical diagnosis.

The most common reasons for referral included arrhythmias (30%) and a family history of sudden death (20%). Of the total cohort of 114 patients: 4 patients (4%) had major cMR findings for ARVC, 13 patients (11%) had minor cMR findings, 2 patients had non-specific cMR findings relating to the right ventricle and 95 patients had a negative cMR. Of the 4 patients who had major cMR findings, 3 (75%) had a positive clinical diagnosis. In contrast, of the 13 patients who had minor cMR findings, only 2 (15%) had a positive clinical diagnosis. Out of the 95 negative patients, clinical details were available for 81 patients and none of them had ARVC. Excluding the 14 patients with no clinical data and final diagnosis, the sensitivity of the test was 100%, specificity 87%, positive predictive value 29% and the negative predictive value 100%.

CMR is a useful tool for ARVC evaluation because of the high negative predictive value as the outcome has a significant impact on the clinical decision-making.