Right ventricular cardiomyopathy similar to Uhl's anomaly with atrial flutter and complete AV block

We report an adult patient with right ventricular cardiomyopathy similar to Uhl's anomaly. In this patient an interesting manifestation is a transient atrial flutter with complete A-V block. Right ventricular cardiomyopathy (arrhythmogenic right ventricular dysplasia) is considered a cause of life-threatening arrhythmia. The right ventricular musculature is partially or totally absent and is replaced by fatty and fibrous tissue. Patients may present with ventricular arrhythmias, right heart failure or asymptomatic cardiomegaly. Only a few cases with atrioventricular conduction disturbance have been reported in the literature by now. We report such a case.