Unusual presentation of a patient with arrhythmogenic right ventricular dysplasia treated with a Glenn shunt

Clinically, arrhythmogenic right ventricular dysplasia (ARVD) usually presents with ventricular arrhythmias, and unusual presentations were reported as acute coronary syndrome, heart failure and electrical storm.Taking all this different presentations and treatments in to account, we report a case of ARVD presenting with central cyanosis and clubbing simulating congenital heart disease. Besides this unusual presentation, the patient underwent also an unusual operation for this kind of abnormality, which cured the cyanosis completely.