A 47-year-old woman diagnosed with arrhythmogenic right ventricular cardiomyopathy, was studied at our institution with palpitations, progressive dyspnoea and cyanosis over a 3-year period. Her haemoglobin saturation was 76.1%. The transthoracic echocardiogram showed that both right atrium and ventricle were dilated, with diffuse akinetic and hypokinetic areas. Radionuclide scintigraphy, angiography and magnetic resonance imaging showed dilated and severe dysfunction of the right ventricle, with depressed ejection fraction (0.16), and bulging at the level of the right ventricular anterior wall areas. Doppler examination revealed a right-to-left interatrial shunt through a patent foramen ovale. Contrast transoesophageal echocardiography confirmed the right-to-left shunting. Considering the clinical symptoms were severe and refractory to medical treatment, she was referred for cardiac transplantation. Unfortunately the patient died suddenly a few weeks later.