DOI: 10.36347/sjmcr.2019.v07i03.021

Pages: 241-244

Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary syndrome characterized by slow but progressive locomotor involvement and cardiomyopathy. Cardiac involvement is primarily conduction defects and dilated cardiomyopathy. Here we report the case of 27 year old man who present preexcitation syndrome as a cardiac involvement of emery dreifuss muscular dystrophy. We present a rare case of a 27 years old patient who consulted for recurrent palpitations, related to ventricular preexcitation discovered at ECG holter. The neuromuscular exam and the electromyography revealed an emery dreifuss dystrophy. Cardiac imaging found a left ventricular non compaction. The patient was treated by low dose of betablocker. The outcome was uneventful. In our knowledge, the present case is the first report described emery dreifuss muscular dystrophy presenting ventricular preexcitation.