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Aim: Diagnosis of early stages of arrhythmogenic right ventricular cardiomyopathy (ARVC) with minimal structural abnormalities is challenging. The purpose of this study was to assess the value of repeated cardiac magnetic resonance imaging (CMR) in patients referred for right ventricular arrhythmias and clinical suspicion of ARVC. Methods and Results: Prospective follow-up study of 18 patients (8 females) studied with CMR for suspected ARVC. Patients with implanted defibrillators (ICD) were excluded. Mean follow-up was 37 ± 16 (12–59) months. Patients were assigned to 2 categories (ARVC likely or ARVC unlikely) according to a CMR-score based on right ventricular abnormalities. Clinical follow-up revealed no disease progression in 17 patients (94%). In 1 patient, an ICD was implanted because of disease progression. Of 9 patients with initial findings suggestive of ARVC, follow-up CMR remained positive in 3 and was diagnosed as normal in 6, mainly due to the inability to confirm the presence of fatty infiltrates at follow-up (5 of 6 patients). Initially, 9 patients had a normal CMR and 8 of those remained normal during follow-up. Conclusion: Repeated CMR after an average follow-up of 3 years was normal in 6 of 9 patients with clinical findings consistent with early stages of ARVC at the time of baseline CMR. Thus, CMR diagnosis of early stage ARVC is difficult and should be made with caution. [ABSTRACT FROM AUTHOR]

Abstract: Background: The value of an implantable cardioverter defibrillator (ICD) for primary prevention in dilated cardiomyopathy (DCM) is unclear, as randomized trials could not show a survival benefit compared to drug therapy. It has not been investigated if patients with a very poor left ventricular function (LVEF) could profit from an ICD. Methods: Consecutive patients with DCM who received an ICD between December 1996 and November 2003 were included in this analysis. Patients were divided in group A (secondary prevention) and group B (primary prevention). Both groups were stratified in subgroups with left ventricular ejection fraction (LVEF) below and above 20%. Results: Fifty eight patients were included (male 50, age 56.4±12.7 years). Follow-up was 34±19 months. There was no difference regarding death (18% vs. 11%), but significant differences (p value <0.05) regarding any adverse events (55% vs. 22%), any ICD intervention (48% vs. 17%) and ICD interventions for life-threatening arrhythmias (27% vs. 0%) between group A and B. LVEF was not predictive for events in group A, whereas in group B only patients with a LVEF <20% had events (p value 0.02). Over time there was an increase of the LVEF of more than 15% determined by echocardiography in 36% of patients, significantly more often in group B. Conclusions: Indication for primary prevention with an ICD in DCM should be made with caution. Larger studies are needed to determine if patients with LVEF of <20% might benefit from an ICD. [Copyright &y& Elsevier]