Summary

We present an uncommon case of a 75-year-old woman who was admitted to our department for the evaluation of her double valvular disease that hid a diagnosis of arrhythmogenic right ventricular dysplasia (ARVD). In the course of preoperative workup, a mass of tissue substituting the right ventricular free wall was identified and documented with transthoracic echocardiography, computed tomography scanning, and nuclear magnetic resonance imaging. This pathology was not identified as ARVD. Before and after surgery (double valve replacement), malignant arrhythmias were observed and treated with an implantable cardioverter-defibrillator. The patient underwent reoperation 24 days later for endocarditis and died in the immediate postoperative period from untreatable ventricular arrhythmias. At autopsy the histological characteristics of ARVD were detected. This report highlights the difficulty in diagnosing ARVD in patients who do not fulfil the McKenna criteria.