Atrial paralysis due to progression of cardiac disease in a patient with Emery-Dreifuss muscular dystrophy

Abstract

We present the progressive nature of the disease in a 26 year-old woman who had suffered from Emery-Dreifuss muscular dystrophy detected at the age of three. In 2002, at the age of 20, due to recurring presyncopal states accompanied by sinus bradycardia and atrioventricular block, she was implanted with a dual chamber pacing system. During testing of the pacing in 2008, permanent electrical atrial stand-still without atria stimulation were detected and the mode of heart stimulation was changed to VVIR. (Cardiol J 2011; 18, 2: 189-193)