Fibrolipomatous dysplasia predominantly involved left heart ventricle: Arrhythmogenic cardiomyopathy/dysplasia is sometimes very copmplicated to diagnose despite complex and long lasting examination. We report a čase of 65 year old woman who was on the basis of prior examination indicated for open heart surgery included aneurysmectomy. Based on transthoracic echocardiography and cardiac magnetic resonance imaging (MRI) included latě enhancement with gadolinium severe post heart attact left ventricle dysfunction was diagnosed. Immediately after open heart surgery patient died due to acute failu-re of the left ventricle. At necropsy cardiac examination surprisingly revealed a lipomatous dystrophy predominantly involving left ventricle. This čase report shows how important is an accurate interpretation of cardiac MRI to determine this rare disorder.

        Key words: arrhythmogenic ventricular cardiomyopathy/dysplasia, magnetic resonance, latě hypercontrast gadolinium imaging.