Cardiac abnormalities and Wolfram (DIDMOAD) syndrome: a case report

Cardiac abnormalities are very rarely reported to be associated with Wolfram (DIDMOAD) syndrome. We report a child who presented with cyanotic heart disease and was operated twice for cardiac lesion. At the age of 6 years, the case developed osmotic symptoms and, during evaluation, diabetes insipidus, optic atrophy and deafness were reported. DIDMOAD syndrome with atrial septal defect, severe pulmonary stenosis and hypoplastic right ventricle is not previously reported. In addition to our case, in a literature survey on all reported cases of DIDMOAD syndrome (more than 700), only 11 cases with cardiac abnormalities have been stated. Common abnormalities found are pulmonary stenosis, followed by tetralogy of Fallot and ventricular septal defects. Impact of DIDMOAD syndrome on cardiovascular development needs to be elucidated.