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

Abstract

AIM:To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) and compare cardiac MR (cMR) findings against clinical diagnosis. METHODS: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. RESULTS: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%.CONCLUSION: 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. Key words: Arrhythmogenic right ventricular cardiomyopathy; Cardiomyopathy; Right ventricular; Arrhythmias; Magnetic resonance imaging; Diagnosis; Implantable cardiac defibrillator Core tip: This study was designed to evaluate the referrals with suspected Arrhythmogenic right ventricular cardiomyopathy (ARVC) and compare the findings of cardiac magnetic resonance imaging (cMR) against clinical diagnosis. Currently the diagnosis depends upon a combination of variety of factors including imaging findings. We evaluated all the referrals in our institution over a 4-year period and found a high sensitivity and specificity of cMR for ARVC diagnosis. We have concluded that cMR is a very useful tool for ARVC evalua- RETROSPECTIVE STUDYSubmit a

Left Ventricular Involvement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Predicts Adverse Clinical Outcomes: A Cardiovascular Magnetic Resonance Feature Tracking Study

“…Left ventricular (LV) or biventricular involvement are increasingly identified in ARVD/C patients 4 . Cardiac magnetic resonance (CMR) is a valuable diagnostic tool for ARVD/C due to the high negative predictive value, which can identify mild LV impairment and thus has a significant impact on clinical decision-making 5,6 . Prior studies have demonstrated that CMR evidence of LV involvement is a strong independent predictor of hard cardiac events in the entire population, including patients with a definite, borderline, or possible ARVD/C diagnosis 7 .…”

Influence of Genotype on Structural Atrial Abnormalities and Atrial Fibrillation or Flutter in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

“…Over the last years, cardiac magnetic resonance (CMR) imaging has gained popularity as the modality of choice for the evaluation of ARVD/C, allowing for noninvasive morphological and functional evaluation, as well as tissue characterization in a single investigation . CMR is especially useful for evaluation of the atria, which are difficult to visualize using other imaging techniques .…”

Arrhythmogenic right ventricular cardiomyopathy in patients with biallelic JUP-associated skin fragility

“…Moreover, the sensitivity of CMRI in children is low because arrhythmic presentations precede structural changes 38 . High negative predictive value of Gadolinium-enhanced CMRI makes it a good choice as a rule-out imaging test for evaluation of structural and functional abnormalities in patients suspected to have ARVC 39 .…”