Arrhythmogenic right ventricular cardiomyopathy/dysplasia (Naxos disease) in a case of rheumatic heart disease – A rare occurrence

We report a case of chronic RHD with severe Rheumatic mitral valve stenosis who had undergone closed mitral commissurotomy (CMC) a few years back. The 20-year-old male continued to be symptomatic with dyspnea and intermittent palpitations even after the procedure (CMC). ECG during one of such an episode revealed ventricular tachycardia, which was promptly cardioverted. His physical examination revealed palmoplantar keratoderma and woolly hair. ECG revealed an epsilon wave in lead V1 and 2D ECHO revealed dilated right atrium and right ventricle, a low LVEF and aneurysmal RVOT. MRI revealed fibrofatty replacement of right ventricular apex and free wall associated with areas of fibrosis and fatty replacement in scattered areas of left ventricle.