Arrhythmogenic right ventricular dysplasia: MR features

Arrhythmogenic right ventricular dysplasia (ARVD) is a heart disease characterized by a total or partial fat replacement of the myocardium. A total of 30 patients were studied with a suspected diagnosis of ARVD. Clinical criteria used for evaluation of ARVD were: (a) ventricular origin arrhythmias with a left bundle branch block configuration, (b) T-wave inversion in the anterior precordial leads, (c) ventricular kinetic alterations observed using echocardiography and angiography and (d) cardiac failure when there are no pathologies attributable to other heart diseases. All patients had serial EKG and echocardiography tests. One third of patients underwent angiocardiography; 7 of 30 had Holter; 7 of 30 had exercise test just to evaluate the effectiveness of the anti-arrhythmic therapy. All patients underwent MRI examination. The following MRI criteria were used: (a) high-intensity areas indicating the fatty substitution of the myocardium, (b) ectasia of the right ventricular outflow tract, (c) dyskinetic bulges, (d) dilation of the right ventricle and (e) enlargement of the right atrium. The diagnosis of ARVD was classified as highly probable for patients manifesting at least three positive criteria, probable with two positive criteria, dubious with one and negative in the absence of all criteria. Highly probable diagnosis of ARVD was made in 8 patients, probable in 4, dubious in 7 and negative in 11. The MRI technique is very effective in the assessment of ARVD. The MRI criteria may be helpful in the diagnosis of this condition.