E-page Original Images: Premature coronary artery disease in a patient with Wolfram syndrome

Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness), is a rare genetic disorder. Here we report a case of premature coronary artery disease (CAD) associated with Wolfram syndrome, which has not been reported before. The patient was a 25-year- old man who had congenital cataracts, optic atrophy; diabetes mellitus, deafness and diabetes insipidus. He had exertional chest pain for 2 months. He had no smoking history. His lipid profile and serum homocysteine levels (7.2 μmol/l) were normal. There was no family history of premature CAD. Cardiovascular examination was unremarkable. Electrocardiogram revealed T wave inversions in inferior leads. Transthoracic echocardiography revealed mild hypokinesia at mid-lateral segment of the left ventricle. Coronary angiography revealed a critical stenosis in the mid-portion of the right coronary artery (RCA) (Video 1. See corresponding video/movie images at www.anakarder.com) and non-critical plaques in the left coronary arterial system (Fig. 1). Critical stenosis in the RCA was successfully opened by a 2.75-mm X 13-mm bare metal stent (Fig. 2).