Total pericardial agenesis mimicking arrhythmogenic right ventricular dysplasia

We report the incidental finding of pericardial agenesis in a patient with electrocardiographic and echocardiographic abnormalities mimicking arrhythmogenic right ventricular dysplasia. The anatomic findings were clearly depicted by cardiac magnetic resonance. The patient was asymptomatic and the clinical relevance of this finding would be raised in case a possible differential diagnosis of right heart overload should be necessary. Diagnosis may be easily suspected from the ECG and echocardiography; MRI will provide definite diagnosis.