Arrhythmogenic right ventricular cardiomyopathy mimics: role of cardiovascular magnetic resonance

Abstract

BackgroundCardiovascular magnetic resonance (CMR) is commonly used in patients with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) based on ECG, echocardiogram and Holter. However, various diseases may present with clinical characteristics resembling ARVC causing diagnostic dilemmas. The aim of this study was to explore the role of CMR in the differential diagnosis of patients with suspected ARVC.Methods657 CMR referrals suspicious for ARVC in a single tertiary referral centre were analysed. Standardized CMR imaging protocols for ARVC were performed. Potential ARVC mimics were grouped into: 1) displacement of the heart, 2) right ventricular overload, and 3) non ARVC-like cardiac scarring. For each, a judgment of clinical impact was made.ResultsTwenty patients (3.0%) fulfilled imaging ARVC criteria. Thirty (4.6%) had a potential ARVC mimic, of which 25 (3.8%) were considered clinically important: cardiac displacement (n=17), RV overload (n=7) and non-ARVC like myocardial scarring (n=4). One patient had two mimics; one patient had dual pathology with important mimic and ARVC. RV overload and scarring conditions were always thought clinically important whilst the importance of cardiac displacement depended on the degree of displacement from severe (partial absence of pericardium) to epiphenomenon (minor kyphoscoliosis).ConclusionsSome patients referred for CMR with suspected ARVC fulfil ARVC imaging criteria (3%) but more have otherwise unrecognised diseases (4.6%) mimicking potentially ARVC. Clinical assessment should reflect this, emphasising the assessment and/or exclusion of potential mimics in parallel with the detection of ARVC major and minor criteria.

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

“…Interestingly we also found evidence of other pathologies in 11% of patients with scans negative for ARVC on imaging criteria, the majority of these were diagnosed as dilated cardiomyopathy. This is in keeping with other studies, which report an incidence of significant other etiologies being diagnosed in 4%-8% of the cases [26,27] . We have also shown that scans positive for ARVC on imaging criteria translate into a high percentage of patients formally fulfilling Task Force criteria for ARVC (75% of cases with a major imaging criteria, 15% with a minor criteria) and that no scans negative for ARVC on imaging criteria were subsequently diagnosed with ARVC.…”

“…Major criteria were found in 4% of the cases and minor criteria were found in 11%. Data from other centers report a detection rate of 3%-10% [26,27] , for unselected cMR referrals for possible ARVC. Therefore our detection rate does not differ significantly from these.…”

“…Due to the high sensitivity and specificity, cMR has also been suggested as a routine method of examination if ARVC is clinically suspected [25] . Apart from excluding ARVC, cMR is also sometimes useful in finding ARVC mimics or other clinically significant findings [26,27] . The requests to rule out ARVC constitute a significant proportion of the total cMR referrals in our institution from the cardiologists.…”

Arrhythmogenic Right Ventricular Cardiomyopathy: From Pathophysiology to Diagnosis and Advances in Management

“…[55][56][57][58] CMR has an important role for identification of phenocopies including DCM, cardiac sarcoid and RV volume overload. 59 Left dominant ARVC may mimic DCM but frequent arrhythmias and subepicardial LGE on CMR favour ARVC diagnosis. 43 Cardiac sarcoid may present with TWI in ECG leads V 1 -V 3 , ventricular arrhythmias and RV structural abnormalities.…”

Echocardiography in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Can the technology survive in the era of cardiac magnetic resonance imaging?

“…Pathologies causing RV volume overload such as intracardiac shunts (e.g. atrial septal defects, anomalous pulmonary venous drainage) can be misinterpreted as possible ARVD/C on standard echocardiography [3], and we often depend on CMR for a more definitive diagnosis.…”